Yoshiyuki Kawashima, Kazushige Ihara, Mieko Nakamura, Tsutomu Nakashima, Satoshi Fukuda, Ken Kitamura
Epidemiological study of mumps deafness in Japan.
Auris Nasus Larynx. 2005 Jun;32(2):125-8. doi: 10.1016/j.anl.2005.01.009. Epub 2005 Apr 7.
Abstract/Text
In Japan, mumps vaccination is optional and the mumps were increased accompanied with prevalent waves according to the Infections Agents Surveillance Report (IASR). The aim of this study was to clarify that there was relevance for increase of mumps epidemic and mumps deafness. The Acute Profound Deafness Research Committee of the Japanese Ministry of Health and Welfare (reorganized to the Ministry of Health, Labour and Welfare in 2001) conducted a nationwide epidemiological survey to determine the number of patients treated for mumps deafness in 1987, 1993 and 2001. Based on its findings, the annual numbers of mumps deafness cases was estimated to be 300 in 1987, 400 in 1993 and 650 in 2001, which correlated with the overall incidence of mumps in those years. Because the majority of cases exhibited severe or profound sensorineural hearing loss that usually did not recover, rapid improvement of mumps vaccine coverage is strongly recommended.
橋本裕美：難治性のムンプス難聴をこれ以上放置すべきではない おたふくかぜワクチンの重要性. 日本小児科医会会報 2008; 35: 129-134.
川島慶之, 佐藤宏昭ほか：平成12・13年度登録の急性低音障害型感音難聴症例の平成19年時点での経過調査(厚生労働科学研究難治性疾患克服研究事業による急性高度難聴に関する調査研究). Audiology Japan 2008；51(3): 200-207.
Tsutomu Nakashima, Taku Hattori, Michihiko Sone, Kiyomitsu Asahi, Naoko Matsuda, Masaaki Teranishi, Tadao Yoshida, Ken Kato, Eisuke Sato
Cochlear blood flow and speech perception ability in cochlear implant users.
Otol Neurotol. 2012 Feb;33(2):165-8. doi: 10.1097/MAO.0b013e318241c0db.
Abstract/Text
OBJECTIVE: The effect of cochlear blood flow (CBF) on speech perception ability in cochlear implant (CI) users has not been reported. We investigated various factors influencing speech perception including CBF in CI users.
PATIENTS: Eighty-two patients who received CI surgery at an academic hospital.
METHODS: CBF was measured during CI surgery using laser Doppler flowmetry. The speech perception level was measured after a sufficient interval after CI surgery. Multivariate analysis was used to evaluate the influences of age, duration of deafness, sex, cause of deafness, and CBF on the speech perception level.
RESULTS: CBF decreased significantly with age but was not related to the speech perception level. In patients with congenital hearing loss, the speech perception level was significantly worse in children who received a CI at 3 years of age than in those who received a CI at 2 years of age or younger. Duration of deafness before CI surgery had deteriorative effects on the speech perception level.
CONCLUSION: CBF may be associated with progression of hearing loss. However, measuring CBF during CI surgery is not useful for predicting postoperative speech perception.
Kristin E D Weimer, Matthew S Kelly, Sallie R Permar, Reese H Clark, Rachel G Greenberg
Association of Adverse Hearing, Growth, and Discharge Age Outcomes With Postnatal Cytomegalovirus Infection in Infants With Very Low Birth Weight.
JAMA Pediatr. 2020 Feb 1;174(2):133-140. doi: 10.1001/jamapediatrics.2019.4532.
Abstract/Text
Importance: Studies suggest that postnatal cytomegalovirus (CMV) infection can lead to long-term morbidity in infants with very low birth weight (VLBW; <1500 g), including bronchopulmonary dysplasia (BPD), necrotizing enterocolitis (NEC), and neurodevelopmental impairment. However, to date, the association of postnatal CMV with hearing, growth, and length of stay among VLBW infants is unknown.
Objectives: To determine the risk for failed hearing screen, increased postnatal age at discharge, or decreased growth at discharge in VLBW infants with postnatal CMV infection compared with CMV-uninfected infants and to compare the risk for other major outcomes of prematurity, including BPD and NEC, in infants with and without postnatal CMV infection.
Participants: This multicenter retrospective cohort study included VLBW infants from 302 neonatal intensive care units managed by the Pediatrix Medical Group from January 1, 2002, through December 31, 2016. Infants hospitalized on postnatal day 21 with a diagnosis of postnatal CMV and hearing screen results after a postmenstrual age of 34 weeks were included in the study population. Data were analyzed from December 11, 2017, to June 14, 2019.
Main Outcomes and Measures: Infants with and without postnatal CMV infection were matched using propensity scores. Poisson and linear regression were used to examine the association between postnatal CMV and the risk of failed hearing screen, postnatal age at discharge, growth, BPD, and NEC.
Results: A total of 304 infants with postnatal CMV were identified, and 273 of these infants (89.8%; 155 boys [56.8%]) were matched with 273 infants without postnatal CMV (148 boys [54.2%]). Hearing screen failure occurred in 45 of 273 infants (16.5%) with postnatal CMV compared with 25 of 273 infants (9.2%) without postnatal CMV (risk ratio [RR], 1.80; 95% CI, 1.14 to 2.85; P = .01). Postnatal CMV was also associated with an increased postnatal age at discharge of 11.89 days (95% CI, 6.72 to 17.06 days; P < .001) and lower weight-for-age z score (-0.23; 95% CI, -0.39 to -0.07; P = .005). Analysis confirmed an increased risk of BPD (RR, 1.30; 95% CI, 1.17 to 1.44; P < .001), previously reported on infants from this cohort from 1997 to 2012, but not an increased risk of NEC after postnatal day 21 (RR, 2.00; 95% CI, 0.18 to 22.06; P = .57).
Conclusions and Relevance: These data suggest that postnatal CMV infection is associated with lasting sequelae in the hearing and growth status of VLBW infants and with prolonged hospitalization. Prospective studies are needed to determine the full effects of postnatal CMV infection and whether antiviral treatment reduces the associated morbidity.
守本倫子、益田慎、麻生伸 他：2015-2016年のムンプス流行時に発症したムンプス難聴症例の全国調査、日本耳鼻咽喉科学会会報. 2018;121: 1173-1180.
P R Donald, E Doherty, F J Van Zyl
Hearing loss in the child following streptomycin administration during pregnancy.
Cent Afr J Med. 1991 Aug;37(8):268-71.
Abstract/Text
The risk of deafness developing in the unborn child following the use of streptomycin (SM) during pregnancy remains uncertain. We have followed up 30 children whose mothers received SM during pregnancy. One child (3pc), whose mother received SM during the first trimester of pregnancy, had profound unilateral hearing loss which could possibly be ascribed to SM. This child had however, in addition, features of congenital hypotonia and a unilateral single crease. Two further children had conductive deafness which could not be due to SM, associated with serious otitis media. Although the risk to the foetus of hearing loss following the use of SM during pregnancy appears relatively low it should, where possible, be avoided during the first trimester of pregnancy.
Tsutomu Nakashima, Taku Hattori, Michihiko Sone, Eisuke Sato, Mitsuo Tominaga, Makoto Sugiura
Blood flow in the ears of patients receiving cochlear implants.
Ann Otol Rhinol Laryngol. 2004 Jun;113(6):426-30.
Abstract/Text
We measured cochlear blood flow (CBF) in 55 patients who received cochlear implants, using a laser-Doppler probe placed over the site of drilling in the cochlear bony wall. The subjects included 29 patients with congenital deafness of unknown cause, 8 with idiopathic progressive sensorineural hearing loss, 4 with postmeningitic deafness, 3 with Waardenburg's syndrome, 3 with congenital cytomegalovirus infection, and 8 whose deafness had other causes. There was a wide range of CBF values in patients with congenital deafness of unknown cause. In the patients with idiopathic progressive sensorineural hearing loss, the CBF was significantly lower in patients more than 40 years old. Intracochlear calcification following meningitis appears to be associated with a reduced CBF.
S Caluwaerts, K VAN Calsteren, L Mertens, L Lagae, P Moerman, M Hanssens, K Wuyts, I Vergote, F Amant
Neoadjuvant chemotherapy followed by radical hysterectomy for invasive cervical cancer diagnosed during pregnancy: report of a case and review of the literature.
Int J Gynecol Cancer. 2006 Mar-Apr;16(2):905-8. doi: 10.1111/j.1525-1438.2006.00223.x.
Abstract/Text
Although cervical carcinoma is among the most frequently encountered malignancies during pregnancy, only four cases of neoadjuvant chemotherapy during pregnancy have been reported. A 28-year-old A0P1G2M0 was diagnosed at 15 weeks with stage Ib1 invasive squamous cervical cancer. Because she strongly desired the continuation of this pregnancy, after extensive counseling she was treated with 75 mg/m(2) cisplatin every 10 days starting at 17 weeks. After six cycles, clinically and radiologically stable disease with normalization of the squamous cell carcinoma tumor marker was obtained. An elective cesarean delivery followed by radical hysterectomy and lymphadenectomy was performed at 32 weeks gestation. The pathology report revealed a moderately differentiated squamous cell carcinoma of 3.5 cm, and all 33 lymph nodes were free of disease. Neonatal examination of the baby could not reveal any abnormalities, and this was confirmed at 6 months. The use of neoadjuvant chemotherapy enabled us to continue this pregnancy until the fetus was viable. Cisplatin did not influence the short-term outcome, but only a long-term follow-up will inform us on its safety during pregnancy.
L Elit, A Bocking, C Kenyon, R Natale
An endodermal sinus tumor diagnosed in pregnancy: case report and review of the literature.
Gynecol Oncol. 1999 Jan;72(1):123-7. doi: 10.1006/gyno.1998.5190.
Abstract/Text
OBJECTIVE: The use of chemotherapeutic drugs in pregnancy is a rare occurrence. Experience and results need to be shared.
METHOD: At 23 weeks gestational age, a patient was diagnosed with an endodermal sinus tumor of the ovary. She received one course of postoperative bleomycin, cisplatin, and etoposide. One week later, the patient was investigated for increasing abdominal pain and the fetus was noted to have ventriculomegaly.
RESULTS: The baby was delivered, and the patient was surgically staged and completed chemotherapy. Although the patient is alive and well 16 months after delivery, the infant has developed significant ventriculomegaly with cerebral atrophy.
CONCLUSION: The etiology of this infant's significant cerebral atrophy is not clear. Tumor-specific, perioperative, or drug-related events must be considered.
Copyright 1999 Academic Press.
Olivier Mir, Paul Berveiller, Stanislas Ropert, François Goffinet, François Goldwasser
Use of platinum derivatives during pregnancy.
Cancer. 2008 Dec 1;113(11):3069-74. doi: 10.1002/cncr.23935.
Abstract/Text
The incidence of cancer during pregnancy is increasing given the trend for women to postpone childbearing. Knowledge of the potential toxicity and teratogenicity of chemotherapy agents is crucial for patient counseling. Platinum derivatives are active against various malignancies that occur more frequently during pregnancy: melanoma, cervical and ovarian cancers, and lung cancer. The authors of this article performed a systematic review of reports documenting the use of platinum derivatives during pregnancy in the English literature from 1977 through January 2008. Forty-three pregnancies were described: 36 patients received cisplatin, 6 patients received carboplatin, and 1 patient received both drugs. Two fetal malformations occurred after in utero exposure to cisplatin, but the causative link between cisplatin administration and these malformations remains speculative. However, either detectable cisplatin levels or platinum-DNA adducts were observed in neonates who were exposed to platinum derivatives during the third trimester, providing evidence for a late-onset transplacental transfer of these drugs. The administration of platinum derivatives, although feasible during the second and third trimesters of pregnancy, raises concern regarding the transplacental transfer of these drugs in late pregnancy and has unknown short- and long-term effects.
(c) 2008 American Cancer Society
Laurell, G. Failure of cisplatin to induce auditory toxicity in guinea pigs exposed in utero. TOXIC SUBSTANCE MECHANISMS 1996; 15(2): 129-133.
Shuai Hao, Xuejun Jiang, Aihui Yan, Bo Yang
Perinatal cisplatin exposure induces cochlear apoptosis in newborn guinea pigs.
Arch Toxicol. 2011 Jan;85(1):19-25. doi: 10.1007/s00204-010-0543-7. Epub 2010 Apr 16.
Abstract/Text
The objective of this study was to evaluate the role of apoptosis in the development of the newborn cochlear structures and hearing loss caused by prenatal cis-diaminedichloroplatinum (cisplatin) exposure. Pregnant albino guinea pigs were intraperitoneally injected with 1.5 mg/kg body weight cisplatin once a day for seven consecutive days at gestational day (GD) 51 to GD 57. At postnatal day (PND) 14, pups were examined in the distortion product otoacoustic emission (DPOAE) task. The temporal bones were then removed and immunohistochemically stained for caspase 3, using the terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) method. Cisplatin used during pregnancy could induce hearing loss in newborn and cochlear hair cell apoptosis. In conclusion, apoptosis may play an important role in the development of hearing impairment, caused by perinatal cisplatin exposure.
American Academy of Pediatrics, Joint Committee on Infant Hearing
Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs.
Pediatrics. 2007 Oct;120(4):898-921. doi: 10.1542/peds.2007-2333.
Abstract/Text
Hironao Otake, Makoto Sugiura, Shinji Naganawa, Tsutomu Nakashima
3D-FLAIR magnetic resonance imaging in the evaluation of mumps deafness.
Int J Pediatr Otorhinolaryngol. 2006 Dec;70(12):2115-7. doi: 10.1016/j.ijporl.2006.07.025. Epub 2006 Sep 15.
Abstract/Text
A 6-year-old boy suffered acute profound right side deafness after his classmates had mumps. Although his salivary glands were not swollen, he had high levels of anti-mumps IgM and IgG antibodies. The three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) procedure applied to magnetic resonance imaging (MRI) showed high signals in the right cochlea and vestibule. This indicated hemorrhage or a high concentration of protein in the right inner ear. This is the first case demonstrating a high 3D-FLAIR MRI signal of the inner ear in a patient with mumps deafness. Our findings suggest that 3D-FLAIR MRI may help to identify and define labyrinthitis in mumps deafness.
Makoto Sugiura, Shinji Naganawa, Seiichi Nakata, Sawako Kojima, Tsutomu Nakashima
3D-FLAIR MRI findings in a patient with Ramsay Hunt syndrome.
Acta Otolaryngol. 2007 May;127(5):547-9. doi: 10.1080/00016480600801399.
Abstract/Text
Three-dimensional, fluid-attenuated inversion recovery (3D-FLAIR) of magnetic resonance imaging (MRI) has recently been developed to detect hemorrhage or high concentrations of protein. We present a patient with Ramsay Hunt syndrome, in whom high signals in the cochlear and vestibular apparatus were identified with 3D-FLAIR. The high signal areas in 3D-FLAIR were not detected by T1- and T2-weighted MRI in this case. This is the first report of high concentrations of protein in the inner ear in Ramsay Hunt syndrome using 3D-FLAIR, and suggests that high concentrations of protein in the inner ear are associated with hearing deterioration in some patients with Ramsay Hunt syndrome. 3D-FLAIR could be a useful diagnostic tool in the early stages of Ramsay Hunt syndrome.
Seiichi Nakata, Terukazu Mizuno, Shinji Naganawa, Makoto Sugiura, Tadao Yoshida, Masaaki Teranishi, Michihiko Sone, Tsutomu Nakashima
3D-FLAIR MRI in facial nerve paralysis with and without audio-vestibular disorder.
Acta Otolaryngol. 2010 May;130(5):632-6. doi: 10.3109/00016480903338123.
Abstract/Text
CONCLUSION: Among patients with facial nerve paralysis, significant difference was observed on three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (3D-FLAIR MRI) between those with and without audio-vestibular disturbance. This MRI technique may contribute to elucidation of the pathology of Ramsay Hunt syndrome and Bell's palsy.
OBJECTIVE: To evaluate the 3D-FLAIR MRI findings in patients who have facial nerve paralysis with and without audio-vestibular disturbance.
METHODS: 3D-FLAIR MRI was performed with and without gadolinium enhancement in 15 patients (5 men and 10 women) with unilateral facial nerve paralysis: 3 patients with Ramsay Hunt syndrome, 3 patients having facial nerve paralysis with hearing loss or vertigo without vesicles, and 9 patients with Bell's palsy.
RESULTS: Five of the six patients with audio-vestibular disturbance showed high signals in the inner ear on precontrast 3D-FLAIR. In comparison, among nine patients with Bell's palsy, only one patient showed high signals in the inner ear on precontrast 3D-FLAIR (p < 0.05).
Michihiko Sone, Terukazu Mizuno, Shinji Naganawa, Tsutomu Nakashima
Imaging analysis in cases with inflammation-induced sensorineural hearing loss.
Acta Otolaryngol. 2009 Mar;129(3):239-43. doi: 10.1080/00016480802226163.
Abstract/Text
CONCLUSION: 3D-FLAIR imaging is sensitive to inflammatory inner ear disturbances and may be a useful method in investigating the severity of inner ear disturbance in cases of inflammation-induced SNHL.
OBJECTIVE: To evaluate the usefulness of the three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) sequence in investigating different etiology of inner ear disturbances in cases with inflammation-induced acute sensorineural hearing loss (SNHL).
PATIENTS AND METHODS: Five cases with inflammation-induced acute SNHL by different conditions are included in this study: acute meningitis, acute otitis media, and Wegener granulomatosis. Imaging analysis was performed using a three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) sequence, and correlation between clinical symptoms and FLAIR abnormalities was evaluated.
RESULTS: In the affected ears in all cases, 3D-FLAIR revealed high pre-contrast signal and increased signal in the cochlea after the administration of gadolinium. Enhancement was still observed in the inner ear after several months with continuing nystagmus in those cases induced by meningitis and severe otitis media. In a case with Wegener granulomatosis, increased signal in the post-contrast images was stronger on the side of the cochlea with the worse hearing level.
Tadao Yoshida, Makoto Sugiura, Shinji Naganawa, Masaaki Teranishi, Seiichi Nakata, Tsutomu Nakashima
Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging findings and prognosis in sudden sensorineural hearing loss.
Laryngoscope. 2008 Aug;118(8):1433-7. doi: 10.1097/MLG.0b013e318172ef85.
Abstract/Text
OBJECTIVES/HYPOTHESIS: Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) has recently been developed to detect high concentrations of protein or hemorrhage. We have previously reported that 50% of patients with sudden sensorineural hearing loss (SNHL) show high signals in the affected inner ear on 3D-FLAIR MRI. However, the relationship between 3D-FLAIR findings and hearing prognosis is unclear. Our objective was to evaluate the relationship between the results of 3D-FLAIR MRI at 3 Tesla and prognosis in sudden SNHL.
STUDY DESIGN AND METHODS: We used 3D-FLAIR at 3 Tesla with and without gadolinium enhancement to evaluate the pathologic conditions in the inner ears of 48 patients with sudden SNHL.
RESULTS: Thirty-one of 48 patients with sudden SNHL showed high signals in the affected inner ear on precontrast 3D-FLAIR. Hearing improvement in patients with high signals in the affected inner ear on precontrast 3D-FLAIR (25 +/- 19 dB) was significantly worse than that in patients with no signal (45 +/- 27 dB; P < .05). Our analysis suggests that high signals in the affected inner ear on precontrast 3D-FLAIR MRI is a new prognostic factor for sudden SNHL.
CONCLUSIONS: 3D-FLAIR findings show that high signals in the cochlea on precontrast 3D-FLAIR are related to a poor hearing prognosis. These signals may reflect minor hemorrhage or an increased concentration of protein in the inner ear, which has passed through blood vessels with increased permeability or has originated in disrupted cells in the inner ear.
S L Garetz, R A Altschuler, J Schacht
Attenuation of gentamicin ototoxicity by glutathione in the guinea pig in vivo.
Hear Res. 1994 Jun 15;77(1-2):81-7.
Abstract/Text
The effect of glutathione co-therapy on the expression of gentamicin ototoxicity was tested in pigmented guinea pigs. The first group of animals was injected with gentamicin (100 mg/kg body weight/day) for two weeks followed by 10 weeks of rest. A second group received glutathione by gastric gavage immediately prior to each gentamicin injection. Two groups of controls were treated either with saline injections or glutathione gavage alone. Auditory brainstem responses, taken at 2-week intervals, revealed a progressive gentamicin-induced hearing loss reaching a 30 to 40 dB threshold shift at 2 kHz, approximately 60 dB at 8 kHz and 80 dB at 18 kHz. Glutathione co-therapy slowed the progression of hearing loss and significantly attenuated the final threshold shifts by 20 to 40 dB. Morphological evaluation confirmed hair cell loss after gentamicin treatment and protection by glutathione. Drug serum levels were assayed at 2 and 7 days of treatment. There were no differences between the gentamicin (mean = 183 micrograms/ml; range, 90 to 300) and the gentamicin/glutathione group (mean = 164 micrograms/ml; range, 80 to 320). Antimicrobial activity of gentamicin was tested against Staphylococcus aureus and Pseudomonas aeruginosa. A 30-fold molar excess of glutathione did not influence the efficacy of gentamicin. These studies suggest that glutathione protects against ototoxicity by interfering with the cytotoxic mechanism.
R Ravi, S M Somani, L P Rybak
Mechanism of cisplatin ototoxicity: antioxidant system.
Pharmacol Toxicol. 1995 Jun;76(6):386-94.
Abstract/Text
The dose and duration limiting toxic effects of cisplatin are ototoxicity and nephrotoxicity. While several studies have attempted to shed some light on the causes of nephrotoxicity, the reasons for ototoxicity induced by cisplatin are poorly understood. Therefore, this investigation was undertaken to delineate the potential mechanisms underlying cisplatin ototoxicity. The role of glutathione (GSH), oxidized glutathione (GSSG) and malondialdehyde levels, and antioxidant enzyme activities [superoxide dismutase, catalase, GSH peroxidase, and GSH reductase] were examined in cochlear toxicity following an acute dose of cisplatin. Male Wistar rats were treated with various doses of cisplatin. Pretreatment auditory brain stem evoked responses (ABR) were performed and then post-treatment ABRs and endocochlear potentials were also performed after three days. Acute cochlear toxicity (ototoxicity) was evidenced as elevated hearing thresholds and prolonged wave I latencies in response to various stimuli (clicks and tone bursts at 2, 8, 16 and 32 kHz) on ABRs. The endocochlear potentials were reduced (50% control) in cisplatin-treated rats as compared to control animals. The rats were sacrificed and cochleae isolated. The GSH, GSSG and malondialdehyde levels, and antioxidant enzyme activities were determined. Cisplatin ototoxicity correlated with a decrease in cochlear GSH [0.45 +/- 0.012 nmol/mg] after cisplatin administration compared to 0.95-012 nmol/mg in control cochleae (P < 0.05). Superoxide dismutase, catalase activities and malondialdehyde levels were significantly increased in the cochleae of cisplatin injected rats. Cochlear GSH-peroxidase and GSH reductase activity significantly decreased after cisplatin administration.(ABSTRACT TRUNCATED AT 250 WORDS)
S H Sha, J Schacht
Antioxidants attenuate gentamicin-induced free radical formation in vitro and ototoxicity in vivo: D-methionine is a potential protectant.
Hear Res. 2000 Apr;142(1-2):34-40.
Abstract/Text
We have recently suggested antioxidant therapy against aminoglycoside-induced hearing loss based on the hypothesis of a redox-active aminoglycoside-iron complex causing ototoxicity. The present study compares seven antioxidants and iron chelators for their ability to attenuate gentamicin-induced free radical generation in vitro and ototoxicity in guinea pig in vivo. Free radical formation by gentamicin was measured by chemiluminescence detection both in a non-enzymatic system in vitro and in cell culture. Deferoxamine, 2,3-dihydroxybenzoate, or salicylic acid suppressed gentamicin-induced luminescence in both tests. This indicated the usefulness of the assay as a screen for potential protectants since these agents had previously been shown to attenuate gentamicin-induced ototoxicity in vivo. Histidine and D-methionine, amino acids with chelating and antioxidant properties, also suppressed gentamicin-mediated luminosity both in vitro and in cell culture. In contrast, the metal chelators succimer (2, 3-dimercaptosuccinic acid (DMSA)) and trientine (N, N'-bis[2-aminoethyl]-1,2 ethanediamine) promoted free radical formation and were excluded from further studies. Histidine and D-methionine were then administered to guinea pigs receiving concurrent treatment with gentamicin (120 mg/kgx19 days). Threshold shifts induced by gentamicin were significantly attenuated by twice-daily injections of D-methionine. Once-daily injections of histidine or D-methionine were less effective, pointing to the importance of pharmacokinetics in antioxidant protection in vivo. The study presents a simple screening system for agents with the potential to attenuate gentamicin-induced hearing loss. It also supports the hypothesis of free radical formation as an underlying cause of gentamicin ototoxicity.
Anna Rita Fetoni, Bruno Sergi, Aldo Ferraresi, Gaetano Paludetti, Diana Troiani
alpha-Tocopherol protective effects on gentamicin ototoxicity: an experimental study.
Int J Audiol. 2004 Mar;43(3):166-71.
Abstract/Text
Gentamicin, acting as an iron chelator, activates membrane lipid peroxidation (MPL) and induces free radical formation, as observed in vitro and in vivo. Antioxidants, such as alpha-tocopherol, are able to suppress MLP, thus attenuating tissue damage. The present study was designed to investigate the possible protective effects of alpha-tocopherol on gentamicin ototoxicity. The study was carried out on albino guinea pigs (250-350 g). The animals were divided into four groups: group A (n = 4), injected with corn oil daily at a dose of 100 mg/kg body weight intramuscularly (IM); group B (n = 10), treated with corn oil at a dose of 100 mg/kg body weight and gentamicin base at a dose of 100 mg/kg body weight (IM); group C (n = 10). treated with gentamicin alone at a dose of 100 mg/kg body weight (IM); and group D (n = 10), treated with gentamicin at the same dose plus alpha-tocopherol acetate at dose of 100 mg/kg body weight (IM). Electrocochleographic recordings were made from an implanted round-window electrode. All animals were treated for 14 days. The compound action potentials (CAPs) were measured at 2-16 kHz at days 0, 10, 14 and 18 after treatment. Changes in cochlear function were characterized as CAP threshold shifts. Morphological changes were analysed by scanning electron microscopy. Gentamicin induced progressive high-frequency hearing loss of 50-60 dB SPL. alpha-Tocopherol co-therapy slowed the progression of hearing loss. The significant loss of outer hair cells (OHCs) in the cochlear basal turn in gentamicin-treated animals was not observed in the cochleas of animals protected with alpha-tocopherol. This study supports the hypothesis that alpha-tocopherol interferes with gentamicin-induced free radical formation, and suggests that this drug may be useful in protecting OHC function from aminoglycoside ototoxicity, thus reducing hearing loss.
Chisato Fujimoto, Tatsuya Yamasoba
Mitochondria-Targeted Antioxidants for Treatment of Hearing Loss: A Systematic Review.
Antioxidants (Basel). 2019 Apr 24;8(4). doi: 10.3390/antiox8040109. Epub 2019 Apr 24.
Abstract/Text
Mitochondrial dysfunction is associated with the etiologies of sensorineural hearing loss, such as age-related hearing loss, noise- and ototoxic drug-induced hearing loss, as well as hearing loss due to mitochondrial gene mutation. Mitochondria are the main sources of reactive oxygen species (ROS) and ROS-induced oxidative stress is involved in cochlear damage. Moreover, the release of ROS causes further damage to mitochondrial components. Antioxidants are thought to counteract the deleterious effects of ROS and thus, may be effective for the treatment of oxidative stress-related diseases. The administration of mitochondria-targeted antioxidants is one of the drug delivery systems targeted to mitochondria. Mitochondria-targeted antioxidants are expected to help in the prevention and/or treatment of diseases associated with mitochondrial dysfunction. Of the various mitochondria-targeted antioxidants, the protective effects of MitoQ and SkQR1 against ototoxicity have been previously evaluated in animal models and/or mouse auditory cell lines. MitoQ protects against both gentamicin- and cisplatin-induced ototoxicity. SkQR1 also provides auditory protective effects against gentamicin-induced ototoxicity. On the other hand, decreasing effect of MitoQ on gentamicin-induced cell apoptosis in auditory cell lines has been controversial. No clinical studies have been reported for otoprotection using mitochondrial-targeted antioxidants. High-quality clinical trials are required to reveal the therapeutic effect of mitochondria-targeted antioxidants in terms of otoprotection in patients.
工田昌也，夜陣紘治：抗酸化剤によるCDDP難聴の治療．耳鼻臨床 2000；93: 533-539.
K A Pussegoda
Genetic variants associated with cisplatin-induced hearing loss.
Clin Genet. 2010 Jul;78(1):33-5. doi: 10.1111/j.1399-0004.2010.01414_2.x.
Abstract/Text
J Lautermann, B Song, J McLaren, J Schacht
Diet is a risk factor in cisplatin ototoxicity.
Hear Res. 1995 Aug;88(1-2):47-53.
Abstract/Text
This study demonstrates that cisplatin ototoxicity depends on dietary factors and correlates with decreased levels of cochlear glutathione and serum albumin. After 12 days of injections, cisplatin (1 mg/kg body weight, s.c.) caused a small hearing loss in guinea pigs fed a regular, full-protein diet (9 +/- 6 dB at 8 kHz and 10 +/- 9 dB at 18 kHz) but a significantly higher hearing loss in animals on a low-protein diet (23 +/- 17 dB at 8 kHz and 32 +/- 23 dB at 18 kHz). Animals on the low-protein diet gained significantly less weight than those on the regular diet, and cisplatin treatment lowered the weight gain in both groups. The low-protein diet also significantly reduced cochlear glutathione levels from 180 +/- 50 to 90 +/- 21 nmol/mg protein and serum albumin from 2.32 +/- 0.04 to 1.75 +/- 0.06 g/dl. Cisplatin treatment tended to decrease glutathione and serum albumin in animals on a full-protein diet but not on the low-protein diet. Renal function was assessed by measuring blood urea nitrogen (BUN) and serum creatinine. While BUN and creatinine values indicated some cisplatin-induced nephrotoxicity, there was no correlation with the severity of ototoxicity. Furthermore, serum platinum levels did not differ between animals on either diet, ruling out a potential influence of altered pharmacokinetics on ototoxicity. These results suggest that the metabolic state of the animal is a risk factor for cisplatin ototoxicity.
S Murakami, N Hato, J Horiuchi, N Honda, K Gyo, N Yanagihara
Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment.
Ann Neurol. 1997 Mar;41(3):353-7. doi: 10.1002/ana.410410310.
Abstract/Text
Although the antiviral agent acyclovir is currently used for the treatment of Ramsay Hunt syndrome, its effects on facial nerve and hearing recovery remain controversial. We retrospectively analyzed the effects of acyclovir-prednisone treatment in 80 Ramsay Hunt patients. Of 28 patients for whom treatment was begun within 3 days of the onset of facial paralysis, the recovery from paralysis was complete in 21 (75%). By comparison, of 23 patients for whom treatment was begun more than 7 days after onset, recovery from facial paralysis was complete in only 7 (30%). A significant difference in facial nerve recovery was found between these groups. Early administration of acyclovir-prednisone was proved to reduce nerve degeneration by nerve excitability testing. Hearing recovery also tended to be better in patients with early treatment. There was no significant difference in facial nerve outcome between intravenous and oral acyclovir treatment.
M Kinishi, M Amatsu, M Mohri, M Saito, T Hasegawa, S Hasegawa
Acyclovir improves recovery rate of facial nerve palsy in Ramsay Hunt syndrome.
Auris Nasus Larynx. 2001 Aug;28(3):223-6.
Abstract/Text
OBJECTIVE: Although the antiviral agent, acyclovir, is currently employed for the treatment in Ramsay Hunt syndrome, the benefit of acyclovir on facial nerve is still unknown and remains controversial. This study was designed to evaluate the effect of acyclovir in facial nerve recovery in Ramsay Hunt syndrome.
METHODS: To evaluate drug effect on facial nerve function, evaluation of the facial voluntary movement and nerve excitability testing were performed. We have used an infusion therapy of acyclovir in combination with a high dose of steroid (AS), which was started within 7 days of onset of facial nerve palsy in 91 patients with Ramsay Hunt syndrome. The results were compared with those of 47 patients whose therapy was steroid alone started within 7 days of onset.
RESULTS: Out of 91 patients treated with AS, nerve exitability was good in 68 (75%), while it was poor in 17 and absent in six. Of 47 patients treated with steroid alone, nerve exitability was good in 25 (53%), while it was poor in 11 and absent in 11. There was statistically significant difference between AS and steroid therapy in the posttreatment degree of nerve function. Complete recovery to grade I in facial voluntary movement was attained in 82 of 91 patients (90%) in the AS therapy, while out of 47 patients treated with steroid alone complete recovery to grade I was attained in only 30 (64%). A statistically significant difference in the recovery rate of facial nerve function was induced between AS and steroid therapy.
CONCLUSION: The AS therapy was proved to keep good degree of nerve function indicated with nerve excitability testing and improve recovery rate of facial nerve in Ramsay Hunt syndrome. Based on this study, we now believe that the AS therapy is an advisable treatment modality to improve the recovery rate of facial nerve function in Ramsay Hunt syndrome.
C J Sweeney, D H Gilden
Ramsay Hunt syndrome.
J Neurol Neurosurg Psychiatry. 2001 Aug;71(2):149-54.
Abstract/Text
The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt's analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome. Compared with Bell's palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely. Studies suggest that treatment with prednisone and acyclovir may improve outcome, although a prospective randomised treatment trial remains to be undertaken. In the only prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy. Further, Bell's palsy is significantly associated with herpes simplex virus (HSV) infection. In the light of the known safety and effectiveness of antiviral drugs against VZV or HSV, consideration should be given to early treatment of all patients with Ramsay Hunt syndrome or Bell's palsy with a 7-10 day course of famciclovir (500 mg, three times daily) or acyclovir (800 mg, five times daily), as well as oral prednisone (60 mg daily for 3-5 days). Finally, some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva. This indicates that a proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete. Treatment of these patients with acyclovir and prednisone within 7 days of onset has been shown to improve the outcome of recovery from facial palsy.
G Nigro, H Scholz, U Bartmann
Ganciclovir therapy for symptomatic congenital cytomegalovirus infection in infants: a two-regimen experience.
J Pediatr. 1994 Feb;124(2):318-22.
Abstract/Text
The efficacy of two regimens of ganciclovir therapy was evaluated in 12 infants with symptomatic congenital cytomegalovirus (CMV) infection. Virologic investigations included culture from urine, saliva, and cerebrospinal fluid, detection of CMV DNA by polymerase chain reaction, and detection of CMV class-specific antibodies (IgG, IgA, IgM) by enzyme immunoassays. Six infants were given ganciclovir, 5 mg/kg twice daily for 2 weeks (group 1); the other six infants were given 7.5 mg/kg twice daily for 2 weeks and 10 mg/kg three times weekly for 3 months (group 2). In group 1 the CMV cultures of specimens from three infants became sterile; two of these infants also had negative results on CMV DNA studies; results of culture and CMV DNA study were still positive after ganciclovir therapy in the remaining three infants. Subsequently, normal outcome was observed in only two patients. In group 2, all infants had negative CMV-culture and CMV DNA results; clinical improvement was evident in five infants, one of whom had later development of mild psychomotor retardation. In another infant, severe psychomotor retardation and hearing loss developed after transient improvement developed. These preliminary data indicate that a ganciclovir regimen including a higher dose and more prolonged therapy might be more effective in infants with symptomatic congenital CMV infection.
Marian G Michaels, David P Greenberg, Diane L Sabo, Ellen R Wald
Treatment of children with congenital cytomegalovirus infection with ganciclovir.
Pediatr Infect Dis J. 2003 Jun;22(6):504-9. doi: 10.1097/01.inf.0000069767.43169.2d.
Abstract/Text
BACKGROUND: Congenital cytomegalovirus (CMV) infection affects approximately 1% of live births in the US. Ten percent of these infants have symptoms at birth and another 10 to 15% acquire hearing loss or developmental problems. Congenital CMV is the most common cause of nonhereditary sensorineural hearing loss in children, and progressive hearing loss is common. To arrest the natural progression of congenital CMV, children referred to our center were treated with a prolonged course of ganciclovir.
METHODS: Medical records of children with congenital CMV who were treated with ganciclovir were reviewed to tabulate their presenting symptoms, duration of treatment, audiologic and developmental assessments and complications.
RESULTS: We treated nine children with symptomatic CMV with iv ganciclovir at a median age of 10 days (range, 3 days to 11 months). Findings at diagnosis included microcephaly (five of nine); petechiae (five of nine); thrombocytopenia (seven of nine); and intracranial calcifications (six of eight). Hearing loss was noted before therapy in five of nine. The median duration of iv and subsequent oral ganciclovir was 1 year and 0.83 year, respectively. Median follow-up was 2 years (range, 1 to 7 years). No child had progression of hearing loss; improvement occurred in two. Seven children had at least one complication of ganciclovir therapy: central venous catheter/site infection (six); catheter malfunction (three); and neutropenia (one).
CONCLUSION: Of nine children none treated with ganciclovir for congenital CMV had detectable progressive hearing loss. Complications associated with iv therapy occurred frequently. Currently available oral analogues of ganciclovir may facilitate earlier and more prolonged therapy for children with symptomatic congenital CMV and should be subjected to randomized controlled trials.
Naoko Tanaka-Kitajima, Naomi Sugaya, Takeshi Futatani, Hirokazu Kanegane, Chizuko Suzuki, Makoto Oshiro, Masahiro Hayakawa, Masahide Futamura, Tsuneo Morishima, Hiroshi Kimura
Ganciclovir therapy for congenital cytomegalovirus infection in six infants.
Pediatr Infect Dis J. 2005 Sep;24(9):782-5.
Abstract/Text
BACKGROUND: Congenital cytomegalovirus (CMV) infection is common, and its morbidity rate is high. Ganciclovir (GCV) treatment has been used for congenital CMV infection, but there are few reports on viral loads associated with GCV therapy.
METHODS: A real-time PCR assay was used to monitor viral load in 6 cases of symptomatic CMV infection that received GCV therapy. Initially GCV was given at a dose of 5-12 mg/kg/d for 2-7 weeks. In 2 cases, additional doses were given as symptoms returned.
RESULTS: After GCV administration, active signs of chorioretinitis, thrombocytopenia and anemia disappeared or improved in all cases. During GCV therapy, viral loads decreased while patients improved clinically and increased again when GCV therapy was stopped. Although CMV DNA continued to be detectable for a long period, clinical findings did not always worsen. In 2 cases, an improvement of hearing loss was observed.
CONCLUSION: GCV therapy transiently suppresses the CMV concentrations. Subsequent increases of viral titers do not appear to be correlated with the clinical course or neurologic outcome.
E P Acosta, R C Brundage, J R King, P J Sánchez, S Sood, V Agrawal, J Homans, R F Jacobs, D Lang, J R Romero, J Griffin, G Cloud, R Whitley, D W Kimberlin, National Institute of Allergy and Infectious Diseases Collaborative Antiviral Study Group
Ganciclovir population pharmacokinetics in neonates following intravenous administration of ganciclovir and oral administration of a liquid valganciclovir formulation.
Clin Pharmacol Ther. 2007 Jun;81(6):867-72. doi: 10.1038/sj.clpt.6100150. Epub 2007 Mar 28.
Abstract/Text
Cytomegalovirus (CMV) is the most common viral congenital infection, producing both sensorineural hearing loss and mental retardation. Our objective was to assess the population pharmacokinetics of a research-grade oral valganciclovir solution in neonates with symptomatic congenital CMV disease. Twenty-four neonates received 6 weeks of antiviral therapy. Ganciclovir and valganciclovir were measured by liquid chromatography/tandem mass spectroscopy. NONMEM version VI beta was used for population analyses. All profiles were consistent with a one-compartment model. Postnatal age, body surface area, and gender did not improve the model fit after body weight was taken into account. The typical value of clearance (l/h), distribution volume (l), and bioavailability of ganciclovir were 0.146 x body weight (WT)(1.68), 1.15 x WT, and 53.6%, respectively. Although these results cannot be extrapolated to extemporaneously compounded valganciclovir preparations, they provide the foundation on which a commercial-grade valganciclovir oral solution may be a viable option for administration to neonates.
David W Kimberlin, Edward P Acosta, Pablo J Sánchez, Sunil Sood, Vish Agrawal, James Homans, Richard F Jacobs, David Lang, Jose R Romero, Jill Griffin, Gretchen A Cloud, Fred D Lakeman, Richard J Whitley, National Institute of Allergy and Infectious Diseases Collaborative Antiviral Study Group
Pharmacokinetic and pharmacodynamic assessment of oral valganciclovir in the treatment of symptomatic congenital cytomegalovirus disease.
J Infect Dis. 2008 Mar 15;197(6):836-45. doi: 10.1086/528376.
Abstract/Text
BACKGROUND: Intravenous ganciclovir administered for 6 weeks improves hearing outcomes in infants with symptomatic congenital cytomegalovirus (CMV) disease involving the central nervous system.
METHODS: Twenty-four subjects received antiviral therapy for 6 weeks. Serial pharmacokinetic assessments were performed after administration of valganciclovir oral solution and of intravenous ganciclovir.
RESULTS: On the basis of a previous pharmacokinetic study of the use of intravenous ganciclovir in this population, a target AUC12 (area under the concentration-time curve over a 12-h period) of 27 mg x h/L was defined. The median dose of oral valganciclovir administered in the present trial was 16 mg/kg, which produced a geometric mean AUC12 of 27.4 mg x h/L. The bioavailability of valganciclovir was 41.1%. Of the 18 subjects who had detectable CMV in whole blood at baseline or during therapy, 11 had <4 log viral DNA copies/mL at baseline, and 7 had > or =4 log viral DNA copies/mL at baseline; subjects who started the study with the higher viral burden experienced greater decreases in viral load but did not clear virus during the 42-day course of therapy. Neutropenia of grade 3 or 4 developed in 38% of subjects.
CONCLUSIONS: In neonates with symptomatic congenital CMV disease, valganciclovir oral solution provides plasma concentrations of ganciclovir comparable to those achieved with administration of intravenous ganciclovir. The results of the present study cannot be extrapolated to extemporaneously compounded liquid formulations of valganciclovir.
井上泰宏：ムンプス難聴. Audiology Japan 2008; 51(6): 617-623.
神崎晶：【小児の耳鼻咽喉科108の疑問】聴覚 ムンプス難聴に治療が必要か? JOHNS 2012;28(3): 308-309.
Jayne M Ramirez Inscoe, Thomas P Nikolopoulos
Cochlear implantation in children deafened by cytomegalovirus: speech perception and speech intelligibility outcomes.
Otol Neurotol. 2004 Jul;25(4):479-82.
Abstract/Text
BACKGROUND: Concerns have been expressed with regard to suitability for cochlear implantation of children deafened by cytomegalovirus because of possible coexisting central disorders/learning difficulties. The aim of the current study was to assess speech perception and intelligibility of speech produced by children deafened by cytomegalovirus and compare their progress with that of congenitally deaf children after cochlear implantation.
METHODS: The study assessed 16 implanted children who were deafened by cytomegalovirus, using the Iowa Closed Sentence Test and Speech Intelligibility Rating. The results were compared with those of a group of 131 children who had undergone implantation who were congenitally deaf but did not have cytomegalovirus as the cause of deafness. The mean age at implantation was 3.9 years for the cytomegalovirus group (median, 3.5 years) and 4.1 years (median, 4 years) for the congenitally deaf children. They all received the Nucleus multichannel cochlear implant system. The follow-up period ranged from 1 to 5 years after implantation for both groups.
RESULTS: After cochlear implantation, the intelligibility of speech produced by children deafened by cytomegalovirus had a wide range, varying from unintelligible speech to connected speech intelligible to all listeners. Relative to the median score for the control group at the last evaluation interval, 3 of the 16 children with cytomegalovirus (19%) performed better, 8 children (50%) performed more poorly, and 5 (31%) performed the same. The difference between the two groups was not statistically significant (p > 0.05). With regard to speech perception Iowa Sentence Test (Level B), relative to the median score for the control group at the last evaluation interval, 5 of the 16 children with cytomegalovirus (31%) performed better, 3 children (19%) performed more poorly, and 8 (50%) performed the same. The difference between the two groups was not statistically significant (p > 0.05). With regard to Level A and relative to the median score for the control group at the last evaluation interval, 1 of the 16 children with cytomegalovirus (6%) performed better, 6 children (38%) performed more poorly, and 9 (56%) performed the same. The difference between the two groups was statistically significant (p = 0.04).
CONCLUSION: The results of the current study showed that cytomegalovirus alone, as a cause of deafness, is not a contraindication for cochlear implantation. Parents should be informed about the wide range of linguistic outcomes after implantation and that these children may need more specific or intensive rehabilitation. Although additional problems are common and outcomes may, on average, be poorer, cochlear implantation can provide useful auditory input to these children. Further research is needed to identify factors associated with cytomegalovirus that may influence the outcomes.
Daniel J Lee, Lawrence Lustig, Margaret Sampson, Jill Chinnici, John K Niparko
Effects of cytomegalovirus (CMV) related deafness on pediatric cochlear implant outcomes.
Otolaryngol Head Neck Surg. 2005 Dec;133(6):900-5. doi: 10.1016/j.otohns.2005.08.013.
Abstract/Text
OBJECTIVE: Human cytomegalovirus (CMV) is a commonly recognized viral cause of perinatal sensorineural hearing loss. CMV-infected infants are also at risk for developmental neurological deficits. This retrospective study assesses the impact of CMV-induced deafness on pediatric cochlear implant outcomes.
STUDY DESIGN AND SETTING: Thirteen patients from the Johns Hopkins pediatric cochlear implant database were identified with CMV-related deafness. A retrospective review of the medical records of the Johns Hopkins Hospital was performed.
RESULTS: The mean age at implantation was 5.6 years. Follow-up audiometric data ranged from 6 to 48 months postoperatively. Mean speech perception scores were 4.5 (out of 6) following implantation.
CONCLUSION: We have shown that cochlear implants can provide useful speech comprehension to patients with CMV-related deafness. Speech recognition scores were within the range established by our overall pediatric implant population.
SIGNIFICANCE: This observation underscores the importance of a multidisciplinary rehabilitation program following implantation in these patients at risk for cognitive delay.
Satoshi Iwasaki, Hiroshi Nakanishi, Kiyoshi Misawa, Toru Tanigawa, Kunihiro Mizuta
Cochlear implant in children with asymptomatic congenital cytomegalovirus infection.
Audiol Neurootol. 2009;14(3):146-52. doi: 10.1159/000171476. Epub 2008 Nov 13.
Abstract/Text
OBJECTIVE: To evaluate the development of speech perception and auditory skills after cochlear implantation in deaf children with asymptomatic congenital cytomegalovirus (CMV) infection diagnosed based on the presence of CMV DNA in the neonatal urine.
STUDY DESIGN: A prospective study of congenital CMV infection was done between 1996 and 2003. Of 18 children diagnosed with congenital CMV infection, 2 deaf children with asymptomatic CMV infections received cochlear implantation.
RESULTS: The 2 deaf children who received cochlear implantation had delayed-onset, progressive sensorineural hearing loss on follow-up audiometric examinations administered at 29 and 39 months of age. After cochlear implantation, their Infant-Toddler Meaningful Auditory Integration Scale scores increased consistently during 36 months of follow-up; these results were similar to those of 5 congenitally deaf children without CMV infection who had cochlear implantation.
CONCLUSIONS: Cochlear implantation was effective for improving the development of speech perception and auditory skills in deaf children with asymptomatic congenital CMV infection. There was no significant difference in the development of useful auditory integration between our general pediatric cochlear implant population without CMV infection and those with asymptomatic CMV infection.
Copyright 2008 S. Karger AG, Basel.
Haruo Yoshida, Yukihiko Kanda, Haruo Takahashi, Ikue Miyamoto, Tomomi Yamamoto, Hidetaka Kumagami
Cochlear implantation in children with congenital cytomegalovirus infection.
Otol Neurotol. 2009 Sep;30(6):725-30. doi: 10.1097/MAO.0b013e3181b1212e.
Abstract/Text
OBJECTIVE: To assess the impact of cochlear implantation (CI) on children with cytomegalovirus (CMV)-induced deafness.
STUDY DESIGN: Retrospective chart review.
PATIENTS: Four children with congenital CMV-related deafness (CMV group) and 17 children with congenital deafness without CMV infection as the cause of deafness (non-CMV group). The age at CI ranged from 2.0 to 3.3 years (mean, 2.6 yr) in the CMV group and from 1.8 to 3.6 years (mean, 2.6 yr) in the non-CMV group. Their follow-up period ranged from 3.0 to 4.3 years (mean, 3.3 yr) in the CMV group and from 1.6 to 4.3 years (mean, 3.3 yr) in the non-CMV group.
METHODS: Evaluation and comparison of preoperative and postoperative hearing levels, motor, social, and language development, Infant-Toddler Meaningful Auditory Integration Scale, and Enjoji Scale of Infant Analytical Development between the 2 groups.
RESULTS: Within 12 months after CI, the mean score of both language perception and production was poorer in the CMV group than in the non-CMV group, and the difference in the language production was statistically significant. However, 12 months after CI, the language perception and production showed good progress at levels similar to that of the non-CMV group.
CONCLUSION: Long-term results of the language perception and production after CI was satisfactory in Japanese children with congenital CMV-related deafness compared with in subjects deafened by other causes. CMV alone, as a cause of deafness, is not likely a contraindication for CI.
Andrea Ciorba, Roberto Bovo, Patrizia Trevisi, Chiara Bianchini, Rosa Arboretti, Alessandro Martini
Rehabilitation and outcome of severe profound deafness in a group of 16 infants affected by congenital cytomegalovirus infection.
Eur Arch Otorhinolaryngol. 2009 Oct;266(10):1539-46. doi: 10.1007/s00405-009-0944-5. Epub 2009 Mar 13.
Abstract/Text
The aim of the study was to characterize the audiological consequences of congenital cytomegalovirus infection (CMV) and to evaluate the outcome of rehabilitation with hearing aids and/or cochlear implant (CI), associated with an adequate speech-language therapy. A retrospective review of data was made from a total of 16 infants, affected by severe to profound hearing loss from congenital CMV infection, referred to a tertiary audiological center for rehabilitation. Audiological evaluation was performed using behavioral audiometry, auditory brainstem responses (ABR) and/or electrocochleography (ECochG). Of the 16 children (median age at diagnosis of hearing loss: 21.33 +/- 0.7 months) with CMV hearing loss, 14 were affected by profound bilateral hearing loss and received a CI, while 2 were affected by bilateral severe hearing loss and received hearing aids. Cochlear implants can provide useful speech comprehension to patients with CMV-related deafness, even if language development is lower when compared to a group of Connexin (Cx) 26+ cochlear-implanted children (eight subjects), matched for age. Congenital CMV infection still represents a serious clinical condition, as well as an important cause of hearing loss in children. More studies have claimed to identify the pathophysiological mechanisms of damage and thus to ensure a better therapeutic approach. Nonetheless, in cases of CMV-deafened babies, the overall outcome of cochlear implantation is good.
Vikas Malik, Iain A Bruce, Stephen J Broomfield, Lisa Henderson, Kevin M J Green, Richard T Ramsden
Outcome of cochlear implantation in asymptomatic congenital cytomegalovirus deafened children.
Laryngoscope. 2011 Aug;121(8):1780-4. doi: 10.1002/lary.21818.
Abstract/Text
OBJECTIVES/HYPOTHESIS: Congenital cytomegalovirus (cCMV) infection is a common cause of sensorineural hearing loss (SNHL). The incidence of SNHL is higher in symptomatic cCMV infants and is usually identified early. By contrast, the incidence of SNHL is lower in children with asymptomatic cCMV, and the hearing loss can be delayed in onset and progressive. The objective was to compare the outcome of cochlear implantation in children deafened by cCMV with a control group of children with implants who do not have the condition.
STUDY DESIGN: Retrospective review of case notes and data base.
METHODS: Retrospective review of 14 children with asymptomatic cCMV who underwent cochlear implantation. Their outcome measures were compared with those of a matched population by using standard assessment tools.
RESULTS: In the study group, the Modified Categories of Auditory Performance (M-CAP) score (range, 1-7) ranged from 2 to 7 (mean, 4.2). In the control group, the M-CAP ranged from 5 to 7 (mean, 6.0). In the study group, the Manchester Spoken Language Development Scale (MSLDS) score (range, 1-10) ranged from 1 to 9 (mean, 5.4). In the control group, the MSLDS ranged from 3 to 10 (mean, 8.1).
CONCLUSIONS: Children with asymptomatic deafness caused by cCMV benefit from cochlear implantation but perform less well than a comparable group of children with implants who do not have cCMV. There is a range of performance in the cCMV group that may relate to the degree of motor or cognitive disabilities.
Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.
