Audiological findings in children aged 2-6 years with isolated speech-language delay.

BACKGROUND: The present study aimed to assess the prevalence and characteristics of hearing loss (HL) among preschoolers with speech-language delay who had passed the universal newborn hearing screening (UNHS). METHODS: This retrospective study included children aged 2-6 years with isolated speech-language delay who were referred to a third-level audiological center for a comprehensive audiological assessment, including otomicroscopy, tympanometry, and click-evoked auditory brainstem responses. RESULTS: A total of 375 speech-language delayed children (285 male and 90 female) were assessed. The mean age was 41±12.4 months. Overall, 133 children (35.5%) were diagnosed with HL: 16 children (12.0%) had sensorineural HL (six unilaterally and 10 bilaterally), six (4.5%) had mixed HL (one unilaterally and five bilaterally), and 111 (83.5%) had conductive HL (19 unilaterally and 92 bilaterally). Regarding the presence of HL, no significant differences were found between males and females (P=0.60) or age groups (P=0.29). Among children with HL, 24 (18.0%) underwent tympanostomy tube placement (all bilaterally), one (0.8%) underwent unilateral cochlear implantation, eight (6.0%) were fitted with hearing aids (one unilaterally and seven bilaterally), two (1.5%) received a bimodal hearing solution (a cochlear implant on one ear and a hearing aid on the other ear), and 98 (73.7%) received no treatment. CONCLUSIONS: Although UNHS and audiological surveillance programs have been implemented over the years, all children with speech-language delay should undergo audiological evaluation. Indeed, the prevalence of undetected HL is not negligible among preschoolers with speech-language delay and requires early diagnosis and prompt treatment.

Combined Presence in Heterozygosis of Two Variant Usher Syndrome Genes in Two Siblings Affected by Isolated Profound Age-Related Hearing Loss.

Sensorineural age-related hearing loss affects a large proportion of the elderly population, and has both environmental and genetic causes. Notwithstanding increasing interest in this debilitating condition, the genetic risk factors remain largely unknown. Here, we report the case of two sisters affected by isolated profound sensorineural hearing loss after the age of seventy. Genomic DNA sequencing revealed that the siblings shared two monoallelic variants in two genes linked to Usher Syndrome (USH genes), a recessive disorder of the ear and the retina: a rare pathogenic truncating variant in USH1G and a previously unreported missense variant in ADGRV1. Structure predictions suggest a negative effect on protein stability of the latter variant, allowing its classification as likely pathogenic according to American College of Medical Genetics criteria. Thus, the presence in heterozygosis of two recessive alleles, which each cause syndromic deafness, may underlie digenic inheritance of the age-related non-syndromic hearing loss of the siblings, a hypothesis that is strengthened by the knowledge that the two genes are integrated in the same functional network, which underlies stereocilium development and organization. These results enlarge the spectrum and complexity of the phenotypic consequences of USH gene mutations beyond the simple Mendelian inheritance of classical Usher syndrome.

Congenital Cytomegalovirus and Hearing Loss: The State of the Art.

In developed countries, congenital cytomegalovirus (cCMV) infection is the most common congenital viral infection, representing the leading non-genetic cause of sensorineural hearing loss (HL). Diagnosis of cCMV infection can be performed by detection of CMV DNA in urine or saliva within 2-3 weeks after birth, or later in dried blood samples on the Guthrie card. Currently, there are many controversies regarding the preventive, diagnostic, and therapeutic approaches to cCMV infection. HL secondary to cCMV is highly variable in onset, side, degree, audiometric configuration, and threshold changes over time. Therefore, it is of paramount importance to perform a long and thorough audiological follow-up in children with cCMV infection to ensure early identification and prompt treatment of progressive and/or late-onset HL. Early cochlear implantation appears to be a valid solution not only for children with bilateral profound HL, but also for those with single-sided deafness, improving localization ability and understanding speech in noisy environments. Moreover, the decision to apply a unilateral cochlear implant in children with cCMV is strengthened by the non-negligible possibility of hearing deterioration of the contralateral ear over time.

Validity of the Italian adaptation of the Hearing Handicap Inventory for the Elderly (HHIE-It).

Objective: The aim of this study was to assess the validity and reliability of the Italian translation of the Hearing Handicap Inventory for the Elderly (HHIE). Methods: A sample of 275 adults, aged > 65 years, filled out the Italian version of the HHIE (HHIE-It) together with the MOS 36-Item Short Form Health Survey (SF-36). Seventy-one participants answered the questionnaire a second time after six weeks. The internal consistency, test-retest reliability, construct and criterion validity were evaluated. Results: Cronbach's alpha was 0.94 and suggested a high internal consistency. There was also a significant intraclass correlation coefficient (ICC) between test and retest scores. In addition, Pearson's correlation coefficient between the two scores was high and significant. High and significant correlation coefficients were also found between the HHIE-It score and the pure tone average threshold of the better ear and between the HHIE-It and the subscales Role-emotional, Social Functioning and Vitality of the SF-36. These latter results indicate good construct and criterion validity, respectively. Conclusions: The HHIE-It maintained the reliability and validity of the English version suggesting its utility for both clinical and research purposes.

Audiological and vestibular symptoms following SARS-CoV-2 infection and COVID-19 vaccination in children aged 5-11 years.

PURPOSE: The present study assessed the prevalence of audio-vestibular symptoms following SARS-COV-2 infection or COVID-19 vaccination among children, comparing the two groups. A further aim was to evaluate whether children with pre-existing unilateral hearing loss were more prone to adverse events. MATERIALS AND METHODS: This retrospective study included children aged 5-11 years with normal hearing or a proven history of unilateral hearing loss who contracted SARS-CoV-2 or received two doses of COVID-19 vaccine. Tinnitus, hyperacusis, aural fullness, otalgia, otorrhea, new-onset hearing loss, vertigo and dizziness were investigated as possible complications of SARS-CoV-2 infection or the COVID-19 vaccine. RESULTS: This study included 272 children (143 boys, 129 girls), with a mean age of 7.8 ± 2.3 years. Among these, 120 were affected by pre-existing unilateral hearing loss. The most common audio-vestibular symptoms reported by children following SARS-CoV-2 infection and COVID-19 vaccination were aural fullness (33/132, 25 %) and dizziness (5/140, 3.6 %), respectively. All symptoms following COVID-19 vaccination resolved within 24 h. Compared to children who received the COVID-19 vaccine, those infected with SARS-CoV-2 had a higher prevalence of tinnitus (p = 0.009), hyperacusis (p = 0.003), aural fullness (p < 0.001), otalgia (p < 0.001), otorrhea (p < 0.001), and vertigo (p = 0.006). Two girls also experienced new-onset unilateral sensorineural hearing loss following SARS-CoV-2 infection. Children with a known history of unilateral hearing loss did not have a higher prevalence of audio-vestibular symptoms than children with normal hearing. CONCLUSIONS: Our results suggest that the COVID-19 vaccine is safe and can be recommended for children with unilateral hearing loss without fear of possible audio-vestibular sequelae.

Hearing outcomes in children with congenital cytomegalovirus infection: From management controversies to lack of parents' knowledge.

OBJECTIVES: Congenital cytomegalovirus (cCMV) is the leading nongenetic cause of sensorineural hearing loss (HL). However, there are no universally accepted approaches to diagnosis, follow-up and treatment. The aim of this study was to evaluate the main characteristics of cCMV-infected children, focusing on their management and long-term hearing outcomes. METHODS: This retrospective study included all children with cCMV infection who were referred to a third-level referral audiologic center for a 6-year hearing follow-up. The main information collected from the medical records included gestational age, birth weight, trimester of maternal seroconversion, hearing status at birth and after 6 years, hearing fluctuations, treatment with oral valganciclovir (within the first month of life and for 6 months), use of hearing devices, presence of speech-language delay, motor delay, cognitive delay and balance disorders, awareness of cCMV among parents, and parents' engagement in behaviors that could increase the risk of CMV infection during pregnancy. RESULTS: A total of 141 children with cCMV infection (72 males and 69 females; mean gestational age: 37+3 weeks; mean birth weight: 2893 g) were assessed. Overall, 48 children (34.0%) had a diagnosis of speech-language delay, 32 (22.7%) of sensorineural HL (59.4% bilaterally; 50% of profound degree), 18 (12.8%) of motor delay, 16 (11.3%) of balance disorders, and 6 (4.3%) of cognitive delay. Among children with HL, 8 (25.0%) were fitted with hearing aids (5 unilaterally and 3 bilaterally), and 5 (15.6%) had undergone cochlear implantation (1 unilaterally and 4 bilaterally), while a bimodal hearing solution was adopted for 2 (6.3%) patients. Compared to children with asymptomatic cCMV infection, symptomatic children had a higher prevalence of neurological and auditory sequelae (P < 0.01) and bilateral (P = 0.003) and severe-to-profound HL (P = 0.004). Overall, 23 children (16.3%) received oral valganciclovir, and only one of them experienced hearing deterioration. Only 14.9% of mothers and 5% of fathers were aware that cCMV could cause progressive or late-onset HL, and 87.9% of parents (248/282) had engaged in behaviors that increased the risk of CMV infection during pregnancy. CONCLUSION: This study confirmed the importance of performing a long audiological follow-up in children diagnosed with cCMV infection due to the possible late-onset, progressive and fluctuating nature of HL. Moreover, the study highlighted many current controversies in preventive (poor prenatal education), diagnostic (routine maternal serological screening) and therapeutic (valganciclovir administered to asymptomatic children) approaches to cCMV infection. More efforts should be made to improve prevention strategies and raise awareness of cCMV infection risks among the population.

Poly-D,L-lactic acid polymer and cochlear implantation.

This case report describes a peculiar and innovative fixing procedure with a Poly-D,L-lactic acid (PDLLA) polymer in the unusual case of magnet dislodgment and rupture of the cochlear implant (CI) silicone sheath holding the magnet.

Impact of COVID-19 containment measures on antibiotic prescribing for respiratory and ear diseases in pediatric primary care.

BACKGROUND: In Italy, the antibiotic prescription rate for children is very high. The aim of this study is to evaluate the change in the monthly rate of antibiotic prescriptions for respiratory and ear infections among children during the COVID-19 pandemic. METHODS: This retrospective study included all children aged <14 years who referred to a pediatric primary care clinic in Milan (Italy) between January 1, 2019, and April 30, 2022. We examined the trends in the antibiotic prescription rate, comparing the pre-lockdown (2019), total lockdown (March-April 2020), partial lockdown (November-December 2020) and post-lockdown (November-December 2021 and March-April 2022) periods. RESULTS: A total of 12 569 visits (mean age of patients: 6.3±3.8 years), with 3228 antibiotic prescriptions, were included in the study. The overall antibiotic prescription rate was 25.7%, with a nadir (5.8%) in May 2020. Compared to 2019, the highest reduction in antibiotic prescription rate was found in February 2021 (-30.3% [95% CI, -36.2%, -24.4%]). A significant increase in the antibiotic prescription rate was observed in November-December 2021 and in March-April 2022 compared to the corresponding periods of the previous year (P<0.001). CONCLUSIONS: This study showed a drastic decrease in the antibiotic prescription rate for respiratory and ear infections during the COVID-19 pandemic, with a nadir immediately after the total lockdown. Day care center and school attendance did not reverse this positive trend during the partial lockdown, unlike what happened with the relaxation of restrictive measures and the resumption of social activities after the lockdown.

Hearing outcomes in preterm infants with confirmed hearing loss.

OBJECTIVES: Premature infants are at high risk for hearing loss (HL). The aim of the present study is to assess the frequency of preterm infants affected by HL who experience hearing improvement over months and evaluate possible factors associated with hearing changes. METHODS: This retrospective study was conducted in a third-level referral audiologic center. Preterm infants with a confirmed diagnosis of sensorineural HL within the first 3 months of life were reassessed at 18 months corrected age using the click-evoked auditory brainstem response between January 1, 2012, and June 30, 2020. The frequency of hearing improvement and associations between possible risk factors and hearing changes were evaluated. RESULTS: A total of 138 preterm infants (71 male and 67 female; mean gestational age: 30+2 weeks) were assessed. The percentages of hearing improvement and hearing threshold normalization were 58.7% (81/138) and 35.5% (49/138), respectively. We observed a higher frequency of hearing improvement among preterm infants who had received exclusive breastfeeding or mixed feeding compared with those who had received exclusive infant formula (80% versus 29.3%, P < 0.001). CONCLUSION: This study confirms the importance of performing a long audiological follow-up and postponing the indication for cochlear implantation in children with a history of preterm birth. Because of the expression of mesenchymal stem cells and high total antioxidant capacity, breast milk might play a protective role in the auditory system of preterm infants. These findings could have important implications for clinical practice, positively impacting the long-term hearing outcomes of preterm infants.

TWNK in Parkinson's Disease: A Movement Disorder and Mitochondrial Disease Center Perspective Study.

BACKGROUND: Parkinsonian features have been described in patients harboring variants in nuclear genes encoding for proteins involved in mitochondrial DNA maintenance, such as TWNK. OBJECTIVES: The aim was to screen for TWNK variants in an Italian cohort of Parkinson's disease (PD) patients and to assess the occurrence of parkinsonism in patients presenting with TWNK-related autosomal dominant progressive external ophthalmoplegia (TWNK-adPEO). METHODS: Genomic DNA of 263 consecutively collected PD patients who underwent diagnostic genetic testing was analyzed with a targeted custom gene panel including TWNK, as well as genes causative of monogenic PD. Genetic and clinical data of 18 TWNK-adPEO patients with parkinsonism were retrospectively analyzed. RESULTS: Six of 263 PD patients (2%), presenting either with isolated PD (n = 4) or in combination with bilateral ptosis (n = 2), carried TWNK likely pathogenic variants. Among 18 TWNK-adPEO patients, 5 (28%) had parkinsonism. CONCLUSIONS: We show candidate TWNK variants occurring in PD without PEO. This finding will require further confirmatory studies. © 2022 Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson Movement Disorder Society.

Maximum acceptable level for the determination of ECAP and ESRT in a paediatric population.

OBJECTIVES: Two of the most used objective measures are electrically evoked action potentials (ECAPs) and electrically evoked stapedius reflex thresholds (ESRTs). Although stimuli used for these measures differ considerably, both measures are influenced by subjective loudness percept. We focus on the subjective maximum acceptable loudness (MAL) to investigate if loudness sensitivity varied along the electrode array during ECAP recordings. In addition, we explored how the MAL reached during an ECAP recording related to the postoperative ESRT. METHODS: Uni- and bilaterally implanted young CI users (n = 15, average age = 9 y, age range 3-18 y) underwent ECAP and ESR recordings using the clinical software MAESTRO (MED-EL, Innsbruck, Austria) and a commercially available immittance instrument (PATH MEDICAL GmbH, Germering, Germany). RESULTS: Loudness tolerance during ECAP recordings was lowest at the two apical-most electrode contacts (number 1 and 2). There was a moderate correlation between the MAL achieved during ECAP recordings and ESR maximum stimulation amplitudes. (r: 0.44344). CONCLUSIONS: ECAP recordings should commence at basal or medial contacts to increase the users' comfort and loudness tolerance, especially in young CI users. A higher maximum stimulation appears to increase the chance of the automatic determination of ECAP thresholds for all electrode contacts.

The Effect of the Use of Hearing Aids in Elders: Perspectives.

Older adults with hearing loss have difficulties during conversation with others because an elevated auditory threshold reduces speech intelligibility, especially in noisy environments. Listening and comprehension often become exhausting tasks for hearing-impaired elders, resulting in social isolation and depression. The aim of the present study was to investigate the advantages of hearing aid use in relation to relief from listening-related fatigue, which is still controversial. Participants included a sample of 49 hearing-impaired elders affected by presbycusis for whom hearing aids were prescribed. The Modified Fatigue Impact Scale was used to assess cognitive, physical and psychosocial fatigue. The vitality subscale of the Short Form Health Survey 36 and a single item of the multi-dimensional Speech, Spatial and Quality Hearing Scale ("Do you have to put a lot of effort to hear what is being said in conversation with others?") were also used. The Cognitive Failures Questionnaire was used to investigate daily errors related to lack of memory and reduced mindedness. Hearing aids rehabilitation resulted in improved speech intelligibility in competing noise, and a significant reduction in cognitive and psychosocial fatigue and listening effort in conversation. Vitality was also improved and a significant reduction in the Cognitive Failures Questionnaire scores was observed. Findings from the study indicate that the use of hearing aids in older impaired-listeners provide them not only with an increased auditory function but also with a reduction in listening-related fatigue and mindedness.

Autologous fat grafting as a minimally invasive technique to avoid cochlear implant extrusion.

ObjectivesInfection of the skin-muscle flap is one of the most severe risks of cochlear implantation. The aim of this paper is to describe a novel and minimally invasive procedure to avoid cochlear implant (CI) extrusion.Methods: A 79-year-old woman with severe comorbidities developed a pressure injury of the skin-muscle flap overlying the CI receiver/stimulator (R/S) nine years after surgery. Skin thinning and failure of conventional pressure injury management led the patient to a high risk of CI extrusion. Therefore, she underwent a single procedure of autologous fat grafting under local anesthesia to increase scalp thickness and vascularization over the CI R/S.Results: Within one month, complete healing of the pressure injury was observed, and the patient was able to safely use her CI. No further signs of infection and skin deterioration were detected at the 15-months follow-up.Discussion: Fat grafting has been proven to promote neoangiogenesis and tissue regeneration. To the best of our knowledge, this is the first report of fat grafting utilized in the skin-muscle flap area to avoid incipient CI R/S extrusion.Conclusion: The described case demonstrates the efficacy of this salvage procedure to avoid major surgery and the additional costs related to CI reimplantation.

Prevalence of symptoms in 1512 COVID-19 patients: have dizziness and vertigo been underestimated thus far?

The relationship between SARS-CoV-2 infection and dizziness is still unclear. The aim of this study is to assess the prevalence and characteristics of dizziness and vertigo among patients with mild-to-moderate COVID-19. Patients discharged from the emergency rooms with a confirmed SARS-CoV-2 diagnosis were assisted by daily telephone calls until nasopharyngeal swab negativization, and specific symptoms concerning balance disorders were investigated through targeted questions posed by experienced physicians. The study included 1512 subjects (765 females, 747 males), with a median age of 51 ± 18.4 years. New-onset dizziness was reported by 251 (16.6%) patients, among whom 110 (43.8%) complained of lightheadedness, 70 (27.9%) of disequilibrium, 41 (16.3%) of presyncope, and 30 (12%) of vertigo. This study analyzed in detail the prevalence and pathophysiological mechanisms of the different types of balance disorders in a large sample, and the results suggest that dizziness should be included among the main symptoms of COVID-19 because one-sixth of patients reported this symptom, with females being significantly more affected than males (20.3 vs 12.9%, P < 0.001). Most cases of dizziness were attributable to lightheadedness, which was probably exacerbated by psychophysical stress following acute infection and mandatory quarantine. Vertigo should not be underestimated because it might underlie serious vestibular disorders, and disequilibrium in elderly individuals should be monitored due to the possible risk of falls.

Impact of Cochlear Implant Array Placement on Speech Perception.

PURPOSE: To assess the role of flat panel computed tomography (FPCT) in the evaluation of cochlear implant (CI) electrode position and its relation to speech perception. METHODS: From March 2015 to March 2019, we retrospectively enrolled deaf subjects ≥ 18 years who underwent unilateral CI by one surgeon, imaged with FPCT and assessed with disyllabic words score before CI and at 6 months of follow-up. We calculated the disyllabic score difference before CI and after CI (ΔSDS) and divided the subjects in favorable and unfavorable outcome groups using the median ΔSDS as a cutoff. We compared the demographic, clinical, electrode characteristics, and the CI positioning variables scalar position, surgical insertion depth (SID), linear insertion depth (LID), angular insertion depth (AID) and wrapping factor (WF). RESULTS: We studied 50 subjects (F/M = 27/23; median age = 60.5 years, IQR: 50-70 years). The median ΔSDS was 80% (interquartile range [IQR]: 60-100%) in quiet and 80% (IQR: 47.5-100%) in noise. Of the subjects 23 demonstrated a favorable outcome and had earlier age at CI (median 52 years; IQR 45-67 years versus median 62 years; IQR: 56-71 years p = 0.032) and a significantly higher SID (median: 4.02 mm IQR: 3.00-5.35 mm versus median: 2.94 mm IQR: 2.06-3.90 mm; p = 0.029). No difference was found for LID (p = 0.977), AID (p = 0.302), and WF (p = 0.224). A logistic regression model built with the age at CI, number of CI electrodes, and the SID was significant χ2 ((df = 3, N = 50) = 14.517, p = 0.002). The model explained 33.7% (Nagelkerke R2) of ΔSDS variance and correctly classified 76% of the cases. CONCLUSION: The SID measured by FPCT predicts the ΔSDS at 6 months follow-up, alongside with age at implantation and number of CI electrodes.

Prognostic Value of Early Magnetic Resonance Imaging Patterns in Sudden Hearing Loss.

INTRODUCTION: Sudden sensorineural hearing loss (SSHL) is a relatively frequent disease, but a sensitive marker or a reliable test to identify the underlying cause is still unavailable. Neuroradiology appears to offer the most promising tools, especially magnetic resonance imaging (MRI). In a recent study from our group, we explored the ability of MRI to detect subtle changes in the inner ear compartments by means of a 3D-fluid-attenuated inversion recovery sequence, aiming at identifying 3 distinct MRI patterns (haemorrhagic, inflammatory, brain-labyrinth barrier breakdown). In the present study, we contrasted the MRI patterns at onset with relevant prognostic factors, with the audiological features of each patient's SSHL and with treatment outcomes. METHODS: In this retrospective study, we enrolled 50 adult subjects (54.61 ± 18.26 years) with SSHL. They underwent an MRI within 72 h from admission, and 5 audiological evaluations: at admission, on the 5th day after the start of medical therapy, at the end of the first cycle of hyperbaric oxygen therapy, then 1 and 6 months later. RESULTS: Abnormalities of the MRI signal and/or post-contrast enhancement asymmetry of the cochlea ("pattern+ MRI") correlated with worse audiological outcomes at 1 month, but the different MRI patterns were not correlated with any specific prognostic model, despite rigid protocol settings. However, a significant difference was found for low-tone SSHL, which were always "pattern" negative at MRI (p = 0.01), and for profound SSHL which demonstrated a pattern+ MRI in 80% (p = 0.04). At the onset of SSHL, a pattern+ MRI was found in 29/50 cases (58.0%) and was related with lesser degree of recovery of pure-tone average at 1 month and lesser chance to retain the hearing threshold benefit in the long term. Given the limited numbers of patients enrolled so far, the relative impact of comorbidities on each MRI pattern remains uncertain. At 6 months, we observed a trend of greater and more stable recovery (p = 0.023) and less frequent recurrence of SSHL in patients with a normal MRI. CONCLUSIONS: The 3 observed MRI patterns did not correlate consistently with specific audio-vestibular features or any peculiar aspect of the patient's clinical history. Larger series of patients with SSHL are needed, possibly from multicentric studies.

A Predictive Model of Bilateral Sensorineural Hearing Loss in Meniere Disease Using Clinical Data.

OBJECTIVES: Meniere disease (MD) is defined by a clinical syndrome of recurrent attacks of spontaneous vertigo associated with tinnitus, aural fullness, and sensorineural hearing loss (SNHL). Most patients have unilateral SNHL, but some of them will develop contralateral SNHL during the course of the disease. Several studies have reported a frequency of 2 to 73% SNHL in the second ear, according to the duration of disease and the period of follow-up. We hypothesize that unilateral and bilateral MD are different conditions, the first would initially involve the apical turn of the cochlea, while bilateral MD would affect the entire length of the cochlea. The aim of the study is to search for clinical predictors of bilateral SNHL in MD to build a predictive model of bilateral involvement. DESIGN: A retrospective, longitudinal study including two cohorts with a total of 400 patients with definite MD was carried out. The inception cohort consisted of 150 patients with MD and the validation cohort included 250 cases. All of the cases were diagnosed of unilateral MD according to their hearing loss thresholds. The following variables were assessed as predictors of bilateral SNHL for the two cohorts: sex, age of onset, familiar history of MD, migraine and high-frequency hearing loss (HFHL, defined if hearing threshold >20 dB in two or more consecutive frequencies from 2 to 8 KHz). A descriptive analysis was carried out according to the presence of HFHL in the first audiogram for the main variables. By using multiple logistic regression, we built-up several predictive models for the inception cohort and validated it with the replication cohort and merged dataset. RESULTS: Twenty-three (19.3%) and 78 (41%) of patients with HFHL developed contralateral SNHL during the follow-up, in the inception and validation cohorts, respectively. In the inception cohort, the best predictive model included HFHL in the first audiogram (OR = 6.985, p = 0.063) and the absence of migraine (OR = 0.215, p = 0.144) as clinical predictors for bilateral SNHL [area under the curve (AUC) = 0.641, p = 0.002]. The model was validated in the second cohort (AUC = 0.621, p < 0.001). Finally, we merged both datasets to improve the precision of the model including HFHL in the first audiogram (OR = 3.168, p = 0.001), migraine (OR = 0.482, p = 0.036) and age of onset >35 years old (OR = 2.422, p = 0.006) as clinical predictors (AUC = 0.639, p < 0.001). CONCLUSIONS: A predictive model including the age of onset, HFHL in the first audiogram and migraine can help to assess the risk of bilateral SNHL in MD. This model may have significant implications for clinical management of patients with MD.