Idiopathic Sensorineural Hearing Loss Research Articles

Clinical Utility of the Standardized Word Recognition Score.

An unexpectedly low word recognition (WR) score may be taken as evidence of increased risk for retrocochlear tumor. We sought to develop evidence for or against using a standardized WR (sWR) score in detecting retrocochlear tumors. The sWR is a z score expressing the difference between an observed WR score and a Speech Intelligibility Index-based predicted WR score. We retrospectively compared the sensitivity and specificity of pure-tone asymmetry-based logistic regression models that incorporated either the sWR or the raw WR scores in detecting tumor cases. Two pure-tone asymmetry calculations were used: the 4-frequency pure-tone asymmetry (AAO) calculation of the American Academy of Otolaryngology-Head and Neck Surgery and a 6-frequency pure-tone asymmetry (6-FPTA) calculation previously optimized to detect retrocochlear tumors. We hypothesized that a regression model incorporating the 6-FPTA calculation and the sWR would more accurately detect retrocochlear tumors. Retrospective data from all patients seen in the audiology clinic at Mayo Clinic in Florida in 2016 were reviewed. Cases with retrocochlear tumors were compared with a reference group with noise- or age-related hearing loss or idiopathic sensorineural hearing loss. Two pure-tone-based logistic regression models were created (6-FPTA and AAO). Into these base models, WR variables (WR, sWR, WR asymmetry [WRΔ], and sWR asymmetry [sWRΔ]) were added. Tumor detection performance for each regression model was compared twice: first, using all qualifying cases (61 tumor cases; 2332 reference group cases), and second, using a data set filtered to exclude hearing asymmetries greater than would be expected from noise-related or age-related hearing loss (25 tumor cases; 2208 reference group cases). The area under the curve and the DeLong test for significant receiver operating curve differences were used as outcome measures. The 6-FPTA model significantly outperformed the AAO model-with or without the addition of WR or WRΔ variables. Including sWR into the AAO base regression model significantly improved disease detection performance. Including sWR into the 6-FPTA model significantly improved disease detection performance when large hearing asymmetries were excluded. In the data set that included large pure-tone asymmetries, area under the curve values for the 6-FPTA + sWR and AAO + sWR models were not significantly better than the base 6-FPTA model. The results favor the superiority of the sWR computational method in identifying reduced WR scores in retrocochlear cases. The utility would be greatest where undetected tumor cases are embedded in a population heavily representing age- or noise-related hearing loss. The results also demonstrate the superiority of the 6-FPTA model in identifying tumor cases. The 2 computational methods may be combined (ie, the 6-FPTA + sWR model) into an automated tool for detecting retrocochlear disease in audiology and community otolaryngology clinics. The 4-frequency AAO-based regression model was the weakest detection method considered. Including raw WR scores into the model did not improve performance, whereas including sWR into the model did improve tumor detection performance. This further supports the contribution of the sWR computational method for recognizing low WR scores in retrocochlear disease cases.

What Is the Role of Sensorineural Hearing Loss in Fabry Disease Screening?

Fabry disease is a rare hereditary lysosomal storage disease caused by the deficiency of alpha-galactosidase A (α-GLA). Although sensorineural hearing loss is common in Fabry disease, there are no studies in the literature that have screened a population with sensorineural hearing loss for Fabry disease. In this study, we aimed to screen a group of patients who were diagnosed with sensorineural hearing loss and underwent a hearing test for Fabry disease. One hundred sixty eight patients who were aged 18-75 years and diagnosed with idiopathic hearing loss between July 2019 and January 2020 were included. In male patients, α-GLA enzyme activity was analyzed. Patients with low enzyme activity were identified and genetic testing was performed for mutations in the GLA gene. In females, only genetic testing was performed. Eighty four women and 84 men were included in the study. α-GLA enzyme activity was low in 11 of the 84 male patients (13%). One out of these 11 patients had a gene mutation for Fabry disease. Moreover, four relatives of this index patient were diagnosed with Fabry disease in family screening. GLA gene mutation was also found in one of the 84 female patients. Consequently, two (1.2%) of our 168 patients were diagnosed with Fabry disease by screening with enzyme activity and genetic testing. Our study showed that screening for Fabry disease in patients with idiopathic sensorineural hearing loss without other specific findings might be a useful strategy for detecting new cases.

The association between COVID-19 vaccination and idiopathic sudden sensorineural hearing loss, clinical manifestation and outcomes.

Previous data demonstrated an increased incidence of Idiopathic Sensorineural Hearing Loss (ISSNHL) in 2021 compared to 2019-2020, suggesting an association with the anti-COVID-19 vaccine. We aimed to assess our center's incidence and compare the clinical manifestations and outcomes of vaccinated vs. unvaccinated patients. A retrospective chart review of all patients diagnosed with ISSNHL during 2021 was conducted and compared to patients who presented in 2018-2020. Patient demographics, audiometry features, vaccination status, and prognosis were evaluated. Throughout 2021, 51 patients were diagnosed with ISSNHL, compared with 31 during 2020, 38 in 2019, and 41 in 2018, demonstrating a 64%, 34%, and 24% increase, respectively. Among patients who presented in 2021, 13 (25.4%) received the anti-COVID-19 vaccine within 30days before their presentation, and 4 received it within 96h. Most presented after receiving the second or third dose. Patient characteristics, audiometry features, and prognosis did not significantly differ between vaccinated and unvaccinated patients. A marked incline was seen in the 2021 ISSNHL incidence at our medical center, of which 25% of cases were within a month post-anti-COVID-19 vaccination. No significant difference was found in clinical manifestations and outcomes between vaccinated and nonvaccinated patients. While other justifications could be sought, an association cannot be ruled out, and further research is needed.

School Hearing Screening With a Portable, Tablet-Based, Noise-Attenuating Audiometric Headset in Rural Nicaragua.

To investigate the utility and effectiveness of a noise-attenuating, tablet-based mobile health system combined with asynchronous telehealth evaluations for screening rural Nicaraguan schoolchildren for hearing loss. Prospective population-based survey. Rural Nicaraguan communities. There were 3,398 school children 7 to 9 years of age. Diagnostic automated and manual audiometry, detailed asynchronous telehealth evaluations. Referral rates, ambient noise levels, and audiometric results as well as hearing loss prevalence, types, and risk factors. Despite high ambient noise levels during screening (46.7 dBA), no effect of noise on referral rates on automated audiometry or confirmatory manual audiometry in those who failed automated testing was seen. The overall audiometric referral rate was 2.6%. Idiopathic sensorineural hearing loss (SNHL) and cerumen impaction were the most common types of hearing loss in this population with an estimated prevalence of hearing loss (all types) of 18.3 per 1,000 children. SNHL was associated with both drug exposure during pregnancy (p = 0.04) and pesticide exposure in the home (p = 0.03). Hearing screening using a tablet-based, noise-attenuating wireless headset audiometer is feasible and effective in rural low-resource environments with moderately elevated ambient noise levels. The referral rate with noise-attenuating headsets was much lower than that previous reports on this population. In addition, manual audiometry resulted in much lower referral rates than automated audiometry. The confirmed hearing loss rate in this study is comparable to reports from other low-income countries that use some form of noise attenuation during screening. Pesticide exposure and drug exposure during pregnancy are potential causes of SNHL in this population.

A Prospective Analysis of the Association Between Sexual Dysfunction and Idiopathic Sensorineural Hearing Loss in Men

A Prospective Analysis of the Association Between Sexual Dysfunction and Idiopathic Sensorineural Hearing Loss in Men

The effect of the COVID-19 pandemic on sudden idiopathic sensorineural hearing loss: A cross-sectional study in a single center

The effect of the COVID-19 pandemic on sudden idiopathic sensorineural hearing loss: A cross-sectional study in a single center

Prevalence of Endolymphatic Hydrops in Cochlear Implant Candidates with Idiopathic Profound Sensorineural Hearing Loss.

To determine the prevalence of endolymphatic hydrops (EH) in cochlear implant (CI) candidates with idiopathic profound sensorineural hearing loss (SNHL) and its influence on the preservation of audiovestibular function after cochlear implantation. Prospective case series. Tertiary referral center. CI candidates with idiopathic progressive SNHL, but without classic EH-associated symptoms. Delayed intravenous gadolinium-enhanced inner ear fluid-attenuated inversion recovery magnetic resonance imaging as well as pure-tone audiograms, video head impulse tests, and vestibular evoked myogenic potentials before and 4 weeks after cochlear implantation. Prevalence of EH before cochlear implantation, audiovestibular function before and after surgery in hydropic and nonhydropic ears. Thirty-two ears in 16 CI candidates were included. Nine ears (28%) with EH were detected. Although preoperative hearing thresholds, utricular function, and semicircular canal function were not different between the two groups, saccular function was reduced in hydropic ears. Ten subjects received a unilateral CI. Of these, 3 (30%) showed EH on the implanted side. There was no difference regarding postoperative hearing loss between the two groups, but the results point toward a higher vulnerability of hydropic ears with respect to loss of otolith function after cochlear implantation. This is the first study showing that EH can be assumed in about one third of CI candidates with idiopathic profound SNHL, but no classic EH-associated symptoms. Preliminary results suggest that EH has no influence on the preservation of cochlear function but could be a risk factor for loss of otolith function after cochlear implantation.

The Otoprotective Effect of Ear Cryotherapy: Systematic Review and Future Perspectives.

This systematic review investigates ear cooling and cryotherapy in the prevention and treatment of inner ear damage and disease, within the context of animal models and clinical studies. A literature search was carried out in the databases Pubmed and Cochrane Library. Ten studies were identified concerning the otoprotective properties of cryotherapy. Nine of these were rodent in vivo studies (mice, rats, gerbils, guinea pigs). One study involved human subjects and investigated cryotherapy in idiopathic sensorineural hearing loss. The studies were heterogeneous in their goals, methods, and the models used. Disorder models included ischemia and noise damage, ototoxicity (cisplatin and aminoglycoside), and CI-electrode insertion. All ten studies demonstrated significant cryotherapeutic otoprotection for their respective endpoints. No study revealed or expressly investigated otodestructive effects. While limited in number, all of the studies within the scope of the review demonstrated some degree of cryotherapeutic, otoprotective effect. These promising results support the conducting of further work to explore and refine the clinical applicability and impact of cryotherpeutics in otolaryngology.

Effect of N-acetyl-cysteine treatment on sensorineural hearing loss: a meta-analysis.

N‐acetyl‐cysteine (NAC) is an efficacious treatment for sensorineural hearing loss in animal models, such as noise‐induced hearing loss (NIHL), however previous research into the effect of NAC on patients with hearing loss produced contradictory results. In this study, we investigated the effect of NAC treatment on sensorineural hearing loss. PubMed, Web of Science and Embase databases were searched in their entirety using the key words: hearing loss, NAC, N‐acetylcysteine, and sensorineural hearing loss. Studies which included assessment of hearing loss with pure‐tone threshold (PTA) data were selected. Eligible studies regarding the effects of NAC treatment on patients with hearing loss were collected by two independent reviewers. A total of 1197 individuals were included from seven published studies. Two studies reported data for a sudden idiopathic sensorineural hearing loss (SISNHL) group. Three studies reported data for a NIHL group. Other studies reported data for drug‐induced hearing loss. The meta‐analysis demonstrated that the overall effect of NAC treatment on sensorineural hearing loss was invalid. However, NAC treatment was linked with improved patient outcomes of hearing tests in cases of sudden hearing loss, but did not prevent hearing loss induced by noise or ototoxicity. However, there is a need for better‐designed studies with larger samples to further prove the correlation between the effect of NAC and hearing loss.

Potential diagnostic biomarkers for early detection of idiopathic sensorineural hearing loss in type 2 diabetes mellitus patients

Background: Sensorineural hearing loss (SNHL) is a hearing loss that accounts for almost 90% of all hearing loss. Various factors can cause this disorder, one of which is diabetes mellitus (DM). However, few studies still discuss biomarkers to detect the incidence of SNHL in patients with DM as early as possible. The purpose of this paper is to determine the potential of biomarkers to detect SNHL in type 2 diabetes mellitus (T2DM) patients. Methods: The author uses a database in the form of PubMed, Cochrane, and ScienceDirect used to search for articles using the Boolean operator "AND" or "OR" using the keywords "hearing loss," "diabetes," "biomarkers," "Prestin," "lipid profile," and "Nox2". A total of 276 studies were obtained, followed by the title and abstract screening and feasibility assessment (eligibility) to get inclusion studies included in the qualitative analysis (systematic study). Result: A total of 10 inclusion studies consisted of 2 cross-sectional studies, 2 case controls, and six preclinical studies with 105 and 101 patients in the cross-sectional and case-control studies, respectively. A total of 5, 3, and 2 studies reported associations between hearing loss and Prestin, Nox2, and lipid profile values, respectively. Conclusion: The biomarkers such as Prestin, Nox2 and lipid profile have a role in the early diagnosis of SNHL, but no related studies discuss T2DM. Therefore, basic and clinical studies are still needed to determine its effectiveness.

ANİ İDİYOPATİK SENSÖRİNÖRAL İŞİTME KAYBINDA PROGNOSTİK FAKTÖRLERİN ANALİZİ

Objective: Sudden idiopathic sensorineural hearing loss (SISHL) is defined as an audiological emergency and although many studies have been conducted on the factors affecting prognosis, there is no consensus yet. The aim of this study is to analyze the prognostic value of clinical and audiological factors in patients with SISHL. Patients and Methods: The data of 210 patients, 118 male and 92 female, who were treated for SISHL, were analyzed retrospectively. Demographic data, audiometry findings, additional symptoms and diseases of the patients were recorded. Degree of hearing loss; were classified as mild (26-40 dB), moderate (41-55 dB), moderate-severe (56-70 dB), severe (71-90 dB) and deep (> 90 dB) according to pure tone audiometry test. The type of hearing loss was determined as descending type, ascending type, midfrequency type and flat type. The patients were divided into three groups as those who started treatment in the first 3 days, between 3-10 days and after 10 days. The effects of age, gender, audiometric findings, additional symptoms and diseases on pre-treatment hearing level, post-treatment hearing level and hearing gain levels were evaluated. Results: The median age of the patients was 46.0 (18.0) years. The most common flat audiogram (54.8%) was seen in the patients. It was observed that patients with flat type audiograms had more hearing loss and less hearing gain. It was determined that hearing gain was higher in patients with severe hearing loss (p < 0.05). There was no significant difference in the pre-treatment and post-treatment hearing levels (p; 0.051 and 0.409, respectively) according to the treatment initiation time, but there was a significant difference in the hearing gain levels (p = 0.005). In patients who started treatment in the first 3 days, the gain was higher than those who started after 4-10 days and 10 days. It was observed that there was no significant difference in pre-treatment and post-treatment hearing levels and hearing gain levels according to gender, affected ear direction, additional findings and presence of diseases. Conclusion: Early initiation of treatment was found to be the most important prognostic factor in SISHL. Raising public awareness for early diagnosis and treatment will reduce the sequelae that may occur due to SISHL.

Cases of Idiopathic Sensorineural Hearing Loss Exhibiting Medium Pattern and Excess Pattern

我々は過去に，漢方治療を試みた突発性難聴例の報告をしたが，いずれも虚証例であった1）。今回，虚実中間証，実証の突発性難聴例を新たに経験した。症例1は57歳男性。3日前からの右耳鳴，難聴を主訴に受診し，右突発性難聴 Grade3b と診断した。虚実中間証で，肝鬱気滞，瘀血を認め，小柴胡湯と桂枝茯苓丸を併用投与した。投与2週間で治癒した。症例2は48歳男性。2日前からの右難聴，めまいを主訴に受診し，右突発性難聴 Grade4a と診断した。実証で，肝鬱気滞，瘀血を認め，大柴胡湯と桃核承気湯を1週間併用投与した。その後，桃核承気湯を桂枝茯苓丸に切り替えて治療継続したが，高音部中心に若干の左右差が残り，著明回復にとどまった。

Improvement of cochleovestibular function in chronic non-inflammatory perilymphatic fistulas of the labyrinth windows: cases from practice

After a brief discussion of the problem of idiopathic sensorineural hearing loss and perilymphatic fistulas of the labyrinth windows, audiometric tests are identified that are informative for the preoperative diagnosis of the latter. Taking into account the own results of surgical treatment, attention is drawn to the feasibility of a wider use of exploratory tympanotomy in cases of suspected occurrence of perilymphatic fistulas of the labyrinth windows.

Otologic Manifestations After COVID-19 Vaccination: The House Ear Clinic Experience.

Objective:With the increasing numbers of COVID-19 vaccinations available there are some reports of new onset of otologic symptoms. We present our experience in recently vaccinated patients over a 30-day time frame.Study Design:Retrospective chart review.Setting:Tertiary otology ambulatory practice.Patients:All patients with available diagnostic codes, COVID-19 questionnaires and clinical notes.Interventions:Observational recordings.Main Outcome Measures:Within the same 30-day time period in 2019, 2020, and 2021, 1.6, 2.4, and 3.8% respectively, of all office visits were for patients with the diagnosis of new onset idiopathic sensorineural hearing loss (SNHL) without other underlying otologic diagnoses. In this time frame in 2021, 30 patients out of the 1,325 clinical visits had new or significantly exacerbated otologic symptoms that began shortly after COVID-19 vaccination. Specifically, 18 patients received Moderna and 12 patients received Pfizer vaccine. Their mean age was 60.9±13.8 years old; 11 were women and 19 men. The mean onset of symptoms was 10.18 ± 9 days post-vaccination. Symptoms included 25 patients (83.3%) with hearing loss, 15 (50%) with tinnitus, eight (26.7%) with dizziness, and five (16.7%) with vertigo. Eleven patients had previous otologic diagnoses, including six patients with Menière's disease, two with autoimmune inner ear disease (AIED), and three having both.Conclusions:There are no definite correlations to the COVID-19 pandemic or vaccination and new or worsened otologic symptoms. Vaccinated patients with new or exacerbated otologic symptoms should be promptly referred for evaluation. Suspected cases of post-vaccination otologic symptoms should be reported to the Center for Disease Control (CDC) vaccine adverse event reporting system (VAERS).

The role of diffusion tensor imaging in idiopathic sensorineural hearing loss: is it significant?

PurposeTo assess the role of diffusion tensor imaging metrics in the evaluation of the microstructural integrity of the central auditory tract in patients with idiopathic sensorineural hearing loss (SNHL), and to compare these patients with healthy controls.Material and methodsThis prospective study, which was conducted upon 30 subjects (21 males, 9 females; age range from 16 to 65 years, mean age 45years) with SNHL proven by audiometric tests. Ten age- and sex-matched healthy volunteers were included as a control group. Patients (n = 30) and volunteers (n = 10) underwent conventional magnetic resonance imaging and diffusion tensor imaging of the brain. Both fractional anisotropy and mean diffusivity (MD) of 3 points along the acoustic tract (inferior colliculus, lateral lemniscus and superior olivary nucleus) were measured bilaterally in all patients and correlated with controls.ResultsMean fractional anisotropy (FA) values were reduced bilaterally at the superior olivary nucleus and/or lateral lemniscus and more significantly at the inferior colliculus of subjects with SNHL in comparison to the volunteers. In patients of unilateral SNHL, similar results were obtained in the contralateral side when compared to controls with statistically significant difference at the 3 regions (p = 0.001). No significant changes were noticed in the MD parameters either in patient or control groups.ConclusionsThe FA value was a valuable non-invasive biomarker in evaluating the subtle microstructural abnormalities of the central auditory tract in idiopathic SNHL and correlated well with hearing impairment.

Ani İdiyopatik Sensorinöral İşitme Kaybında Sistemik İmmün İnflamasyon İndeksinin Değerlendirilmesi

Objective: This study aimed to investigate the prognostic value of systemic immune inflammation index (SII) in sudden idiopathic sensorineural hearing loss (SISNHL). Methods: Eighty-nine patients diagnosed with SISNHL were included in this study. SII was calculated from the complete blood count when the patients were hospitalised. The patients were treated in accordance with our routine SISNHL protocol. Hearing recovery was evaluated in accordance with Siegel’s criteria, and two main groups were created (complete and incomplete recovery groups). The control group consisted of patients who were admitted to our clinic for hearing examination. SIIs were statistically analysed amongst these three groups (control, complete recovery and incomplete recovery groups). Results: The mean SII of the three groups significantly differed (p= 0.002). The control group significantly differed from the two other groups (complete recovery group: p= 0.023, incomplete recovery group: p= 0.048). The complete recovery group did not significantly differ from the incomplete recovery group (p= 0.950). Conclusion: SII had no prognostic value in patients diagnosed with SISNHL. However, SIIs were significantly higher in the patient groups diagnosed with SISNHL than those in the control group. This finding supports the predictive value of SII in SISNHL. Studies with more participants are needed to demonstrate the predictive value of SII in SISNHL.

İdiyopatik Tek Taraflı Sensörinöral İşitme Kayıplı Hastalarda Kohlear Sinir Çapının Manyetik Rezonans Görüntüleme ile Değerlendirilmesi

Objective: The aim of this study was to evaluate the cochlear nerve diameter in patients with unilateral sensorineural hearing loss and to compare the diameter of the normal side with the hearing loss side. Materials and Methods: A total of 36 patients with idiopathic unilateral sensorineural hearing loss were included in the study. Magnetic resonance imaging (MRI) and audiogram were performed on all the patients. Both cochlear nerve diameters were measured on axial thin-section constructive interference in steady state (CISS) sequences. Results: The patients comprised 16 males and 20 females with a mean age of 52.3±13.7 years. Hearing loss was in the right ear in 11 patients, and in the left ear in 25. The average cochlear nerve diameter on the side with hearing loss was 0.13 cm while it was 0.19 cm on the normal side. There was a statistically significant difference between the two sides (p < 0.001). Conclusion: In patients with unilateral sensorineural hearing loss, MRI allows the assessment of the cochlear nerve with anatomic detail, and enables the exclusion of possible organic pathologies. Demonstration of decreased cochlear nerve diameter in idiopathic sensorineural hearing loss will contribute to elucidating the etiology of this disease in the future.

Different treatment protocols for moderate idiopathic sudden sensorineural hearing loss

Treatment of idiopathic sensorineural hearing loss (ISSNHL) is problematic due to the unclear etiology of the illness. Corticosteroid is recommended by some papers, and hyperbaric oxygen (HBO2) by others. Recently HBO2 has been shown to be an important therapy for ISSNHL, with an increasing number of studies demonstrating its beneficial results. Recovery from ISSNHL depends on the interval period between onset and treatment, hearing loss severity and audiogram type used to determine damage. Treatment of ISSNHL requires a detailed analysis. In this retrospective study we reviewed data from 56 patients with moderate ISSNHL. These patients were divided into three groups based on different treatments: corticosteroid group; corticosteroid + HBO2 (combination)group; and HBO2-only group. Additionally, all patients received intravenous vasodilator treatment. Hearing levels before and after treatment were compared. All three groups had a similar recovery rate, with an effective rate of more than 50%, and a hearing gain average of 17.38 decibels (dB). HBO2 treatment got a higher recovery rate. The combination therapy, which included corticosteroid and HBO2, did not elevate the recovery rate.

Intratympanic Triamcinolone Acetonide as Treatment Option for Idiopathic Sudden Sensorineural Hearing Loss.

Corticosteroids represent the most commonly used treatment option for patients with idiopathic sudden sensorineural hearing loss. In the past, these compounds were mainly formulated and tested for intravenous or oral administration. Intratympanic application is increasingly being used, often as salvage treatment. The most suitable corticosteroid for local application has yet to be identified. Trials have suggested that triamcinolone acetonide has superior molecular properties for this treatment modality. The main aim of this study was to retrospectively assess the first audiometric results of patients diagnosed with idiopathic sudden sensorineural hearing loss and treated simultaneously with systemic prednisolone and intratympanic triamcinolone acetonide. This data was then compared to systemic treatment only, as well as to historic cohorts treated intratympanically with widely used corticosteroids, namely dexamethasone or methylprednisolone. 90 patients received intravenous prednisolone only, and 89 individuals underwent intravenous treatment combined with three to four simultaneous intratympanic applications of triamcinolone. Eight patients received intratympanic triamcinolone as first-line treatment. After adjusting data for sex, time since onset, age, and severity of hearing loss, no statistically significant difference between the two main treatment groups could be identified. No major adverse events were observed, specifically no otitis media or persistent vertigo. Two perforated tympanic membranes healed spontaneously within several days. While the exact role of intratympanic injections requires additional trials, triamcinolone resulted in similar outcomes compared to studies using dexamethasone or methylprednisolone. Due to favorable pharmacological properties, triamcinolone represents a safe and efficacious alternative for intratympanic treatment in idiopathic sensorineural hearing loss.

Prediction of the Outcome of Cochlear Implantation in the Patients with Congenital Cytomegalovirus Infection based on Magnetic Resonance Imaging Characteristics.

The goal of this study was to elucidate radiologic biomarker that can predict the outcome of cochlear implantation (CI) in congenital cytomegalovirus (cCMV) related deafness. A retrospective survey of speech perception after CI and an evaluation of brain magnetic resonance imaging (MRI) findings were performed in 10 cochlear implantees with cCMV-related prelingual deafness. Specifically, a special attention was paid to the degree of white matter (WM) abnormality shown in brain MRI, which was used to divide our cohort into two groups: The mild and severe pathology groups. Age-matched prelingual deaf patients with idiopathic sensorineural hearing loss were selected as controls. Subjects in mild pathology groups showed higher a Category of Auditory Performance (CAP) score (5.2 ± 0.8) than those with severe pathologies (3.4 ± 1.5) (P = 0.041). Importantly, speech performance from subjects with mild pathology was comparable to that of the control group (mean CAP score of 5.2 ± 0.8 vs. 5.1 ± 1.2) (P = 0.898). Mild pathologies related to the limited WM lesion in MRI not accompanied by severe MRI pathologies, such as diffuse WM abnormality, myelination delay, ventriculomegaly, migration abnormality, and cerebellar hypoplasia, can be tolerated and do not adversely affect the CI outcome in cCMV deafness.