Etiology Of Hearing Loss Research Articles

Postoperative Auditory Progress in Cochlear-Implanted Children With Auditory Neuropathy.

This study aims to evaluate the effect of auditory neuropathy spectrum disorder (ANSD) on postoperative auditory perception and listening difficulties in pediatric cochlear implant (CI) recipients. The Children's Auditory Perception Test (CAPT) assesses auditory perception skills, and the Children's Home Inventory of Listening Difficulties (CHILD) Scale evaluates daily listening difficulties. The study involved pediatric CI recipients (n = 40) aged between 5 and 7 years, with and without diagnosis of ANSD. The research ensured homogeneity across various factors, including chronological age, age at diagnosis, age at initial implantation, bilateral simultaneous surgery, etiologies of hearing loss, and family education level. The findings have demonstrated that children without ANSD exhibited better performance in integrating visual-auditory stimuli and overall listening performance, distant sound source scores, and noisy environment scores (respectively p = .047, p = .001, p = .028, and p = .010). Additionally, children with better speech perception also have a better ability to integrate audiovisual stimuli (p = .005, r = .438). There are significant differences in postoperative listening skills and auditory perceptions between children with and without an ANSD who have CIs. Accordingly, children without an ANSD perform better.

A novel frameshift mutation in the DIAPH1 gene causes a Chinese family autosomal dominant nonsyndromic hearing loss: Mutation in DIAPH1 causes hearing loss.

This study reports a novel heterozygous, likely truncating mutation in the diaphanous homolog 1 (DIAPH1) gene associated with non-syndromic hearing loss. Family members underwent audiological and imaging assessments, whole-exome sequencing (WES), and Sanger sequencing. Sensorineural hearing loss was observed in all five individuals, with severity ranging from mild to severe. None of the affected patients reported vestibular complaints, and routine blood tests showed normal platelet counts. Whole-exome sequencing (WES) revealed a novel frameshift variation, c.3555delA (p.Gln1185Hisfs*3), in exon 26 of the DIAPH1 gene. This variation co-segregated with the hearing-impaired phenotype in the family. The data collected support the classification of c.3555delA as a genetic etiology of hereditary hearing loss according to the American College of Medical Genetics and Genomics guidelines. We identified a novel pathogenic mutation in the DIAPH1 gene, thereby expanding the mutation spectrum associated with hearing loss.

A Frameshift Variant in ANKRD24 Implicates Its Role in Human Non-Syndromic Hearing Loss.

Hearing loss (HL) is the most prevalent sensorineural disorders, affecting about one in 1000 newborns. Over half of the cases are attributed to genetic factors; however, due to the extensive clinical and genetic heterogeneity, many cases remain without a conclusive genetic diagnosis. The advent of next-generation sequencing methodologies in recent years has greatly helped unravel the genetic etiology of HL by identifying numerous genes and causative variants. Despite this, much remains to be uncovered about the genetic basis of sensorineural hearing loss (SNHL). Here, we report an Iranian consanguineous family with postlingual, moderate-to-severe autosomal recessive SNHL. After first excluding plausible variants in known deafness-causing genes using whole exome sequencing, we reanalyzed the data, using a gene/variant prioritization pipeline established for novel gene discovery for HL. This approach identified a novel homozygous frameshift variant c.1934_1937del; (p.Thr645Lysfs*52) in ANKRD24, which segregated with the HL phenotype in the family. Recently, ANKRD24 has been shown to be a pivotal constituent of the stereocilia rootlet in cochlea hair cells and interacts with TRIOBP, a protein already implicated in human deafness. Our data implicate for the first time, ANKRD24 in human nonsyndromic HL (NSHL) and expands the genetic spectrum of HL.

Correlation between the etiology of severe hearing loss and endolymphatic hydrops.

This study aimed to investigate correlation between the presence of endolymphatic hydrops(EH) and factors such as causes of hearing loss, patient age, duration of deafness, and results of vestibular function tests. We retrospectively reviewed medical charts of 128 ears of cochlear implantees who were not considered relevant to Meniere's disease. When comparing group with genetic variants of GJB2, SLC26A4, LMX1A and other genetic mutation group, the proportion of vestibular EH and cochlear EH found in group with genetic variants of GJB2, SLC26A4, LMX1A was significantly higher than group with other genetic etiology (p < 0.01) or the group with all the other causes of hearing loss (p < 0.01). The rate of vestibular and cochlear EH detection was higher in younger patients (41.5% and 35.4%) than in older patients (25.4% and 20.6%). A higher ratio of vestibular and cochlear EH was observed in patients with a longer duration of deafness (37.5% and 31.3%) than those with a shorter duration of deafness (29.7% and 25.0%). The group with vestibular EH showed a higher incidence of abnormal findings in the caloric test (42.9%) than the group without vestibular EH (28.2%). Patients with genetic variants of GJB2, SLC26A4, LMX1A, younger patients, those with longer deaf durations showed a higher prevalence of vestibular and cochlear EH, implying EH appears to be formed as a developmental disorder in association with a certain set of genetic variants, rather than a phenotypic marker as a result of severe to profound hearing loss.

Validating the splicing effect of rare variants in the SLC26A4 gene using minigene assay

BackgroundThe SLC26A4 gene is the second most common cause of hereditary hearing loss in human. The aim of this study was to utilize the minigene assay in order to identify pathogenic variants of SLC26A4 associated with enlarged vestibular aqueduct (EVA) and hearing loss (HL) in two patients.MethodsThe patients were subjected to multiplex PCR amplification and next-generation sequencing of common deafness genes (including GJB2, SLC26A4, and MT-RNR1), then bioinformatics analysis was performed on the sequencing data to identify candidate pathogenic variants. Minigene experiments were conducted to determine the potential impact of the variants on splicing.ResultsGenetic testing revealed that the first patient carried compound heterozygous variants c.[1149 + 1G > A]; [919–2 A > G] in the SLC26A4 gene, while the second patient carried compound heterozygous variants c.[2089 + 3 A > T]; [919–2 A > G] in the same gene. Minigene experiments demonstrated that both c.1149 + 1G > A and c.2089 + 3 A > T affected mRNA splicing. According to the ACMG guidelines and the recommendations of the ClinGen Hearing Loss Expert Panel for ACMG variant interpretation, these variants were classified as “likely pathogenic”.ConclusionsThis study identified the molecular etiology of hearing loss in two patients with EVA and elucidated the impact of rare variants on splicing, thus contributing to the mutational spectrum of pathogenic variants in the SLC26A4 gene.

Advances in the Study of Etiology and Molecular Mechanisms of Sensorineural Hearing Loss.

Sensorineural hearing loss (SNHL), a multifactorial progressive disorder, results from a complex interplay of genetic and environmental factors, with its underlying mechanisms remaining unclear. Several pathological factors are believed to contribute to SNHL, including genetic factors, ion homeostasis, cell apoptosis, immune inflammatory responses, oxidative stress, hormones, metabolic syndrome, human cytomegalovirus infection, mitochondrial damage, and impaired autophagy. These factors collectively interact and play significant roles in the onset and progression of SNHL. The present review offers a comprehensive overview of the various factors that contribute to SNHL, emphasizes recent developments in understanding its etiology, and explores relevant preventive and intervention measures.

Cochlear implantation in syndromic patients: difficulties and lessons learnt.

Identify the prevalence of syndromes in a cohort of patients who underwent cochlear implantation, to report on the presence of inner and middle ear malformations and highlight the surgical difficulties encountered. Observational, retrospective study. Tertiary referral children's hospital pediatric cochlear implant program. An IRB-approved retrospective chart review of children undergoing cochlear implantation at a tertiary academic medical center, from 2018 to 2023. Preoperative imaging data of syndromic patients in that cohort with special attention to the presence of inner ear or middle ear malformations were collected. Abnormal intraoperative findings and difficulties reported by the surgeons were also noted. 1024 children were unilaterally implanted for bilateral profound hearing loss. There were 45 cases diagnosed with associated syndromes (4.3%). The commonest syndromes were Jervell and Lange Nielsen (JLN) syndrome followed by Waardenberg syndrome (WS), in a prevalence of 34% and 32% respectively. These syndromes had no associated inner ear malformations (IEM). Less common syndromes included Branchio-oto-renal (BOR) syndrome, CHARGE association and Treacher Collins syndrome, 3 cases each, and keratosis icthyosis deafness syndrome (KID), Usher syndrome and Albino, 2 cases each and an H syndrome case. There were 9 cases (20%) with IEM, with 6 cases of perilymph gusher. Two cases had middle ear anomalies and one case had a facial nerve course abnormality. The outcome of these cases was similar to non-syndromic cases. Children with syndromic HL should be dealt with on a case by case scenario to diagnose inner and middle ear malformations. Additional disabilities can affect the rehabilitation procedures. All children with congenital hearing loss should undergo pediatric, cardiologic, ophthalmologic and nephrologic examination in order to exclude the syndromic etiology of hearing loss.

Pharmacological Approaches to Hearing Loss.

Hearing disorders pose significant challenges to individuals experiencing them and their overall quality of life, emphasizing the critical need for advanced pharmacological approaches to address these conditions. Current treatment options often focus on amplification devices, cochlear implants, or other rehabilitative therapies, leaving a substantial gap regarding effective pharmacological interventions. Advancements in our understanding of the molecular and cellular mechanisms involved in hearing disorders induced by noise, aging, and ototoxicity have opened new avenues for drug development, some of which have led to numerous clinical trials, with promising results. The development of optimal drug delivery solutions in animals and humans can also enhance the targeted delivery of medications to the ear. Moreover, large genome studies contributing to a genetic understanding of hearing loss in humans combined with advanced molecular technologies in animal studies have shown a great potential to increase our understanding of the etiologies of hearing loss. The auditory system exhibits circadian rhythms and temporal variations in its physiology, its vulnerability to auditory insults, and its responsiveness to drug treatments. The cochlear clock rhythms are under the control of the glucocorticoid system, and preclinical evidence suggests that the risk/benefit profile of hearing disorder treatments using chronopharmacological approaches would be beneficial. If translatable to the bedside, such approaches may improve the outcome of clinical trials. Ongoing research into the molecular and genetic basis of auditory disorders, coupled with advancements in drug formulation and delivery as well as optimized timing of drug administration, holds great promise of more effective treatments. SIGNIFICANCE STATEMENT: Hearing disorders pose significant challenges to individuals and their overall quality of life, emphasizing the critical need for advanced pharmacological approaches to address these conditions. Ongoing research into the molecular and genetic basis of auditory disorders, coupled with advancements in drug delivery procedures and optimized timing of drug administration, holds the promise of more effective treatments.

Genetic landscape of hearing loss in prelingual deaf patients of eastern Iran: Insights from exome sequencing analysis.

Hearing loss is one of the most prevalent genetic disorders in humans. Locus and allelic heterogeneity cause fundamental challenges in hearing loss genetic diagnosis and management of patients and their families. This study examined the genetic profile of patients with prelingual hearing loss who were referred to the Genetic Foundation of Khorasan Razavi spanning over a decade. Deleterious variants in GJB2 were evaluated through Sanger sequencing among 745 non-syndromic hearing loss patients. Furthermore, exome sequencing was applied in 250 patients with negative GJB2 sequencing results and 30 patients with syndromic hearing loss. The findings revealed a relatively low frequency of GJB2 variants among the studied patients. Exome sequencing successfully identified the genetic causes of hearing loss in 70% of the patients. Moreover, variants in 10 genes, namely SLC26A4, MYO15A, TMPRSS3, TMC1, OTOF, CDH23, PJVK, MYO7A, TECTA, and PCDH15, accounted for 66% of the positive exome sequencing findings in this study. At least three prevalent founder alleles in the hearing-impaired population of eastern Iran were identified. This study emphasizes the efficiency of exome sequencing as a powerful tool for determining the etiology of prelingual hearing loss in the eastern Iranian population.

First report of an Ivorian family with nonsyndromic hearing loss caused by GJB2 compound heterozygous variants.

The primary etiology of congenital hearing loss is attributed to genetic factors, with GJB2 identified as a pivotal gene across diverse ethnic groups. Additionally, nonsyndromic hearing loss is predominantly inherited in an autosomal recessive manner. We used Sanger sequencing to analyze GJB2 in 17 deaf children from 13 unrelated Ivory Coast families. One family had two children born with severe congenital deafness and exhibited pathogenic compound heterozygous variants. These variants included a nonsense substitution (c.132G>A or p.Trp44Ter) and a newly discovered duplication of 7 base pairs (c.205_211dupTTCCCCA or p.Ser72ProfsTer32). Segregation testing confirmed these variants, marking the first identification of GJB2 in an Ivorian family with congenital hearing loss.

Investigation of Targeted Genes and Identification of Novel Variants with Next Generation Sequencing Method in Hearing Loss.

Hearing loss is a widespread condition throughout the world. It may affect patients from newborns to the elderly. There are too many reasons for hearing loss, including congenital hearing loss, virus infections, age-related situations, and traumatic situations, which may be related to the immune-mediated system. Fifty percent of hearing loss is related to genetic mutations and defects; genetic causes are highly heterogeneous, so the analysis of new variants are important for diagnosis. We aimed to describe the importance of detected gene variations by using targeted gene panels in the Next-Generation-Sequencing (NGS) platform. Eighty-one hearing loss targeted genes were investigated using Illumina NextSeq550 technology in 100 participants with hearing loss between 2017 and 2022 in our Genetic Diseases Evaluation Center. Targeted genes were performed on 100 patients with hearing loss diagnosis. The total number of detected variants was 77. Forty-seven cases have likely pathogenic/pathogenic variants. Thirty of them have uncertain clinical significance variants, and from the detected variants, 8 are novel. In this research, we highlighted that earlier detection of hearing loss using molecular genetic methods may help us understand the etiology and orient for a better prognosis. Results detected by using the NGS platform can assist and improve the diagnosis. In this study, the diagnostic rate with targeted genes was detected as 35.29%. It has an important role in clinical practice as the recommendation of cochlear implants. Clarifying the genotype and phenotype correlation helps us figure out the etiology of hearing loss and also the worth of genetic counseling in hereditary hearing loss.

Impact of nutrition on hearing loss.

The incidence of hearing disorders is rising at an alarming rate, aggravated by increasing longevity, without sufficient social awareness and attention from public health officials. The etiology of hearing loss (HL) is multifactorial, including both genetic and environmental factors, such as noise, ototoxic drugs, and nutritional status. Therefore, it is necessary to expand knowledge about the relationship between the different factors involved as a first step towards prevention and potential repair of hearing damage before it becomes irreversible and significantly limits the quality of life. Our recent studies have demonstrated the interrelationship between nutrition and HL highlighting the strong need to implement prevention and nutritional intervention strategies aimed at promoting hearing health from the earliest stages of life.

Factors impacting outcomes of cochlear implantation in children at two University centres in China: Multi-year analysis from the Paediatric Implanted Recipient Observational Study (P-IROS)

Objectives To identify factors affecting functional hearing performance and quality of life (QoL) outcomes in paediatric cochlear implantation (CI) recipients at two University centres in mainland China. Methods Two university centres in mainland China, part of the prospective longitudinal Paediatric Implanted Recipient Observational Study (P-IROS), contributed participant data. Participants were aged under 10 years at time of CI. Functional hearing performance and QoL measures were collected prior to device activation, and at 6-monthly intervals for 2 years post-implantation. Functional hearing endpoints including Categories of Auditory Performance-II (CAP-II) and QoL were evaluated and analysed using ordinal mixed-effects regression models. Results Data were from 288 children with a mean age at implant of 2.74 years. Overall follow-up at 1 year was 59% and 51% at 2 years. Younger age at implantation (p<0.001) and hearing aid use preimplantation (p=0.026) were associated with significant benefit. Bilateral device users (both CI and bimodal) achieved significantly better functional hearing performance on the CAP-II than unilateral CI users (p<0.001). Slower functional hearing improvements were observed in those with lower parental expectations compared to higher expectations (p<0.001). QoL improved over time but followed a different initial trajectory between centres. Conclusion All participants demonstrated significant improvements in auditory performance and QoL over time. Younger age at CI, and bilateral/bimodal device fitting contributed to earlier improvements. Other potential factors that could help inform families, professionals, and health authorities about choice of hearing device and educational supports required included aetiology of hearing loss and level of maternal education.

Exome sequencing reveals pathogenic mutations in the LARS2 and HSD17B4 genes associated with Perrault syndrome and D-bifunctional protein deficiency in Moroccan families.

Syndromic hearing loss (SHL) is characterized by hearing impairment accompanied by other clinical manifestations, reaching over 400 syndromes. Early and accurate diagnosis is essential to understand the progression of hearing loss and associated systemic complications. In this study, we investigated the genetic etiology of sensorineural hearing loss in three Moroccan patients using whole exome sequencing (WES). The results revealed in two families Perrault syndrome caused by LARS2, p. Asn153His; p. Thr629Met compound heterozygous variants in two siblings in one family; and p. Thr522Asn, a homozygous variant in two sisters in another. The patient in the third family was diagnosed with D-bifunctional protein deficiency (D-BPD), linked to compound heterozygous mutations p. Asn457Tyr and p. Val643Argfs*5 in HSD17B4. Molecular dynamic simulation results showed that Val643Argfs*5 does not prevent HSD17B4 protein from binding to the PEX5 receptor, but further studies are recommended to verify its effect on HSD17B4 protein functionality. These results highlight the effectiveness of WES in identifying pathogenic mutations involved in heterogeneous disorders and the usefulness of bioinformatics in predicting their effects on protein structure.

Postural stability at activation and deactivation of the cochlear implant in adolescents with late lateral implantations: a quasi-experiment

BackgroundCochlear implantation (CI) surgery has become a prevalent method of hearing rehabilitation, since it has been acknowledged that it impacts effectively on the vestibular system. However, there is still no consensus among clinicians on the most appropriate age and area (lateral/bilateral) of CI surgery in terms of postural control. The present study aimed to assess the postural control in late lateral CI adolescents with different visual (eyes opened(EO)/eyes closed(EC)) and auditory (CI activated/deactivated) conditions and to build a theoretical model of postural control based on sensual compensatory mechanisms that are predominant in late CI individuals. It was hypothesized that kinesthetic sensation and exteroceptors of the superficial sensation are critical for neuromuscular control after late CI.MethodsA quasi-experimental study protocol was used in this study to assess the postural stability performance in the studied adolescents with different visual and auditory perceptions. 27 adolescent students with hearing loss participated in the study. A force plate (Accu Gait AMTI) with computer software (NetForce) was used in the study to assess the postural stability with four different conditions(EO)/EC), CI activated/deactivated).ResultsvCOP was found to have a significant growing tendency within the conditions of CI activated/deactivated.No statistically significant relationships were noted between the range of the displacement of feet pressure (Area) and both the visual and auditory conditions. Hearing loss etiology was statistically significantly related to the values of vCOP, within the conditions of EO, CI activated/deactivated (p < 0.01), what did not occure with the condition of EC (p > 0.05). Neuromuscular control with the condition of EC x CI deactivated was found to be based on the kinesthetic-tactual compensatory model.ConclusionsKinesthetic sensation and exteroceptors of the superficial sensation seem to be the predominant source of information to maintain postural control in late CI adolescents, regardless of the visual and auditory conditions. The etiology of hearing loss (congenital/acquired) can be a predictor of the values of the vCOP. In order to improve neuromuscular control in this population, it is recommended that the patients perform physical activity tasks, especially to develop core muscles, based on direct stimulation and rotational stability.

Cochlear reimplantation outcomes over 20 years: Expertise in reimplantation surgery and auditory-speech rehabilitation

ObjectivesThe aim of this study was to present an institution's experience with cochlear reimplantation (CRI), to assess surgical challenges and post-operative outcomes and to increase the success rate of CRI. Study designRetrospective single-institution study. SettingTertiary medical center. MethodsWe retrospectively evaluated data from 76 reimplantation cases treated in a tertiary center between 2001 and 2022. Clinical features including etiology of hearing loss, type of failure, surgical issues, and auditory speech performance were analyzed. Categorical Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were used to evaluate pre- and post-CRI outcomes. ResultsThe CRI population comprises of 7 patients from our institute,69 referred patients from other centers. Device failure was the most common reason (68/76, 89.5 %) for CRI; in addition, there were 7 medical failures and 1 had both soft device failure. Medical failures included flap rupture and device extrusion, magnet migration, auditory neuropathy, leukoencephalopathy, foreign-body residue and meningitis. In 21/76 patients, the electrode technology was upgraded. The mean time to failure was 0.58–13 years, with a mean of 4.97 years. The mean (± SD) CAP and SIR scores before and after CRI were 5.2 ± 1.2 versus 5.5 ± 1.1 and 3.4 ± 1.1 versus 3.5 ± 1.1, respectively. Performance was poor in six patients with severe cochlear malformation, auditory nerve dysplasia, leukoencephalopathy, and epilepsy. ConclusionCRI surgery is a challenging but relatively safe procedure, and most reimplanted patients experience favorable postoperative outcomes. Medical complications and intracochlear damage are the main causes of poor postoperative results. Therefore, adequate preoperative preparation and atraumatic CRI should be carried out for optimal results.

MRI-confirmed cochlear artery infarct clinically diagnosed in a patient with sickle cell disease: a case report.

To corroborate the vascular etiology of sudden sensorineural hearing loss (SNHL) utilizing magnetic resonance imaging (MRI). A 24-year-old male with a history of sickle cell disease experienced sudden SNHL and right horizontal nystagmus, without accompanying vertigo. Audiometric evaluation revealed left-sided SNHL, predominantly affecting high frequencies. Video head impulse testing demonstrated isolated dysfunction of the left posterior semicircular canal. An urgent brain MRI identified a recent punctiform ischemic stroke in the frontal region. A subsequent MRI, conducted with a 4-hour delay and post-contrast enhancement, highlighted a hyperintense signal within the left cochlear region and the left posterior semicircular canal. The investigative results substantiate an infarction in the territory of the cochlear artery, precipitated by a vaso-occlusive event, thereby reinforcing the vascular hypothesis of cochleovestibular artery syndrome. This case underscores the congruence between clinical observations and delayed post-contrast MRI findings.

High prevalence of m.1555A > G in patients with hearing loss in the Baikal Lake region of Russia as a result of founder effect

Mitochondrial forms account approximately 1–2% of all nonsyndromic cases of hearing loss (HL). One of the most common causative variants of mtDNA is the m.1555A > G variant of the MT-RNR1 gene (OMIM 561000). Currently the detection of the m.1555A > G variant of the MT-RNR1 gene is not included in all research protocols. In this study this variant was screened among 165 patients with HL from the Republic of Buryatia, located in the Baikal Lake region of Russia. In our study, the total contribution of the m.1555A > G variant to the etiology of HL was 12.7% (21/165), while the update global prevalence of this variant is 1.8% (863/47,328). The m.1555A > G variant was notably more prevalent in Buryat (20.2%) than in Russian patients (1.3%). Mitogenome analysis in 14 unrelated Buryat families carrying the m.1555A > G variant revealed a predominant lineage: in 13 families, a cluster affiliated with sub-haplogroup A5b (92.9%) was identified, while one family had the D5a2a1 lineage (7.1%). In a Russian family with the m.1555A > G variant the lineage affiliated with sub-haplogroup F1a1d was found. Considering that more than 90% of Buryat families with the m.1555A > G variant belong to the single maternal lineage cluster we conclude that high prevalence of this variant in patients with HL in the Baikal Lake region can be attributed to a founder effect.

Prevalence, Pattern, and Etiology of Hearing Loss in a Tertiary Otolaryngology Center in Central Yemen

Abstract Background and Aims: Hearing loss (HL) is a significant public health concern. Research on its prevalence and pattern are crucial for understanding its causes, identifying high-risk populations, developing prevention strategies, and ultimately, improving the quality of life for people with HL. Therefore, this study aims to investigate the prevalence, pattern, and causes (etiology) of HL among patients attending our ENT Hospital. Study Design: This study was a retrospective review of existing medical records of patients from a Tertiary ENT Hospital in Yemen to determine the prevalence, patterns, and causes of HL among patients attending ENT centers. Results: A total of 951 patients were included in this study. The prevalence of HL was found to be 29.7%. The most commonly affected age group was 11–20 years old, with conductive HL being the most frequent subtype 65.1%. Sensorineural HL followed at 27.8%, and mixed HL was present in 7.1% of patients. Chronic suppurative otitis media (CSOM) 29.5% was identified as the most common cause of HL, followed by otitis media with effusion and wax impaction. Conclusion: HL is a significant public health concern in developing countries. CSOM is a major contributor to HL in this population, and age plays a role in the type of HL experienced.

Middle Ear Fibrosis Contributes to Hearing Loss in Patients With Myhre Syndrome.

Myhre syndrome (MS) is a rare genetic condition that presents with multiple genetic anomalies including cleft lip and palate and Eustachian tube dysfunction. These patients are at a high risk for airway scarring from intubation and mucosal inflammation. Hearing loss (conductive or mixed, of varying severity) is a common comorbidity in these patients, the exact etiology of which is still unclear. We present the cases of 2 unrelated children with MS who suffered progressive mixed hearing loss from fibrosis and obliteration of the middle ear spaces. Both patients had multiple sets of ear tubes that demonstrated early extrusion. The older patient underwent bone conduction implantation at age 11 which resulted in dramatic improvement of speech recognition and interactive skills. The other younger patient demonstrates a similar trajectory but has not yet undergone implantation. Otolaryngologists should take a cautious approach to surgery of the eardrum and middle ear to avoid unnecessary induction of fibrosis in this susceptible patient population. These cases highlight a newly described etiology for hearing loss and suggest a benefit to bone conduction implantation.