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.

Prognostic factors in pediatric cochlear implant: an outcome-based study.

OBJECTIVE: To determine the prognostic factors in the pediatric cochlear implant (CI) outcome. MATERIALS AND METHODS: This prospective cohort study was conducted on 289 pediatric cases with prelingual hearing loss who received cochlear implantation. Several possible salient factors have been recorded. Auditory and speech evaluations were performed before CI, as well as 6 and 12 months after surgery, using Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) tests. RESULTS: According to univariate analysis, age at the time of surgery was a statistically significant factor. Neurological problems in the child, history of newborn infectious diseases, history of hearing aid use, proper parental cooperation, and round window approach were all significantly related to better auditory or speech outcomes. On the other hand, good parental cooperation and age (for CAP) and good parental cooperation, age, history of infectious disease, and hearing aids use (for SIR) are the significant factors in the multivariate setting. CONCLUSION: As evidenced by the obtained results, age, background diseases, history of rehabilitation with hearing aids, and surgical details are essential factors to be taken into account in the case-selection process.

Investigation of MYO15A and MYO7A Mutations in Iranian Patients with Nonsyndromic Hearing Loss.

BACKGROUND: Hearing loss (HL) is the most common sensory disorder in humans, which affects individuals in both inherited and acquired forms. MYO15A and MYO7A gene mutations have a significant role in the development of deafness. In this study, we assessed the prevalence of MYO15A and MYO7A mutations in one hundred non-relative deaf Iranians. Materials and methods: The existence of MYO15A and MYO7A mutations were assessed using the tetra-primer ARMS-PCR method, High Resolution Melting (HRM) and sequencing method. Results: A heterozygote missense mutation, p.V2135L (c.6403G > T) in the MYO15A gene, was found in a patient using the sequencing method. Conclusion: These results explain the negligible prevalence of selected mutations among Iranian patients. Identifying common mutations in patients of an ethnic group can reduce the financial costs and time needed for identifying the causes of deafness.

The conserved p.Arg108 residue in S1PR2 (DFNB68) is fundamental for proper hearing: evidence from a consanguineous Iranian family.

BACKGROUND: Genetic heterogeneity and consanguineous marriages make recessive inherited hearing loss in Iran the second most common genetic disorder. Only two reported pathogenic variants (c.323G>C, p.Arg108Pro and c.419A>G, p.Tyr140Cys) in the S1PR2 gene have previously been linked to autosomal recessive hearing loss (DFNB68) in two Pakistani families. We describe a segregating novel homozygous c.323G>A, p.Arg108Gln pathogenic variant in S1PR2 that was identified in four affected individuals from a consanguineous five generation Iranian family. METHODS: Whole exome sequencing and bioinformatics analysis of 116 hearing loss-associated genes was performed in an affected individual from a five generation Iranian family. Segregation analysis and 3D protein modeling of the p.Arg108 exchange was performed. RESULTS: The two Pakistani families previously identified with S1PR2 pathogenic variants presented profound hearing loss that is also observed in the affected Iranian individuals described in the current study. Interestingly, we confirmed mixed hearing loss in one affected individual. 3D protein modeling suggests that the p.Arg108 position plays a key role in ligand receptor interaction, which is disturbed by the p.Arg108Gln change. CONCLUSION: In summary, we report the third overall mutation in S1PR2 and the first report outside the Pakistani population. Furthermore, we describe a novel variant that causes an amino acid exchange (p.Arg108Gln) in the same amino acid residue as one of the previously reported Pakistani families (p.Arg108Pro). This finding emphasizes the importance of the p.Arg108 amino acid in normal hearing and confirms and consolidates the role of S1PR2 in autosomal recessive hearing loss.

Middle ear function in sinonasal polyposis.

Nasal airway patency has long been considered a major factor in ear health. The aim of this study was to determine the effect of sinonasal polyposis on middle ear and eustachian tube (ET) functionality. Forty-four individuals with polyposis, 23 with non-polyposis nasal obstruction, and 23 healthy controls were enrolled. Demographic, clinical and imaging data of all participants were collected and ET function tests and audiologic tests were performed. Hearing loss (p = 0.02), flat tympanogram (p = 0.02), disturbed Toynbee and Valsalva tests (p = 0.01), and the prevalence of allergy (p = 0.04) and purulent nasal discharge (p < 0.001) were significantly higher in the polyposis group than the other groups. Regression analysis revealed that infection and allergy have more important roles in ET function than the nasal obstruction. Polyposis could impede ET function; however, it is probably not because of its obstructive nature, but because of the associated increased risk of infection.

Hearing disorders in Turner's syndrome: a survey from Iran.

Turner syndrome (TS) is one of the most frequently encountered sex-linked chromosomal abnormalities, occurring in one per 2,000 female births. These patients present with short stature and failure to begin puberty. In this syndrome, there are multiple organ abnormalities, including auditory disorders. TS patients were referred to the ENT clinic by a pediatric endocrinologist. A questionnaire was filled out and the patients went through a complete otologic examination. They were then referred to the audiology clinic to undergo audiologic test battery plus high-frequency pure tone audiometry. From a total of 48 ears examined, 11 (22.9 %) had a normal audiometry. Mid-frequency sensorineural hearing loss (SNHL), high-frequency SNHL, combined and mixed hearing loss were diagnosed in 6 (12/5 %), 20 (41/7 %), 6 (12/5 %) and 1 (2/1 %) ear, respectively. Tympanogram results showed normal compliance (A, As, Ad) in the majority of cases. B and C patterns were found in a few cases. Speech discrimination score was normal in all patients whereas speech reception threshold was normal in 92 % of the ears. Audiometry abnormality especially SNHL is common in TS patients, with the high-frequency pattern being the most frequent.

Accuracy of high-resolution computed tomography in locating facial nerve injury sites in temporal bone trauma.

In this study, high-resolution, multislice computed tomography findings are compared with surgical findings in terms of the fracture location in patients with traumatic facial paralysis. Patients with traumatic facial paralysis with grade VI House-Brackmann scale who met the criteria for surgical decompression between 2008 and 2012 were included in this study. All the patients underwent a multislice high-resolution, multislice computed tomography (HRCT) using 1-mm-thick slices with a bone window algorithm. The anatomical areas of the temporal bone (including the Fallopian canal) were assessed by CT and during the surgery (separately by the radiologist and the surgeon), and fracture line involvement was recorded. Forty-one patients entered this study. The perigeniculate area was the most commonly involved region (46.34 %) of the facial nerve. The sensitivity and specificity of HRCT to detect a fracture line seems to be different in various sites, but the overall sensitivity and specificity were 77.5 and 77.7 %, respectively. Although HRCT is the modality of choice in traumatic facial paralysis, the diagnostic value may differ according to the fracture location. The results of HRCT should be considered with caution in certain areas.

Cochlear Implantation in Children with Meningitis: A Multicenter Study on Auditory Performance and Speech Production Outcomes.

BACKGROUND: This study aims to evaluate speech production outcomes and auditory performance in children with post-meningitis deafness who were treated with cochlear implants. Additionally, the study assesses the impact of electrode insertion depth on surgical outcomes.". METHODS: We conducted a study on 66 pediatric patients with bilateral postmeningitis hearing loss who were being prepared for cochlear implantation at four tertiary referral academic institutions. The speech intelligibility rating (SIR) and categories of auditory performance (CAP) were evaluated after the first and second years following implantation. The patients were divided into two groups based on electrode insertion depth: one group had full electrode insertion (more than two-thirds), while the other had partial electrode insertion (less than two-thirds). We compared the SIR and CAP scores between the two groups to assess the impact of electrode insertion depth on outcomes. RESULTS: Before implantation, the median CAP score was one, but it improved significantly to six within two years after the procedure (P-value < 0.001). Similarly, the median SIR score before implantation was one, but it improved significantly to three within two years after surgery (P-value < 0.001). However, there was no significant difference between the partial and full electrode insertion groups in terms of CAP and SIR scores during the follow-up evaluations conducted after the first and second years. CONCLUSION: The study found that cochlear implantation significantly improved speech production skills and auditory performance in children with postmeningitis deafness. Importantly, the amount of electrode insertion at the time of implantation did not have a significant impact on the outcomes.

Use of a sternocleidomastoid myocutaneous flap based on the sternocleidomastoid branch of the superior thyroid artery to reconstruct extensive cheek defects.

INTRODUCTION: Reconstruction of head and neck defects has always been challenging due to functional and cosmetic concerns. Although sternocleidomastoid (SCM) flaps have been used for many head and neck defects, use of an SCM flap to reconstruct a cheek defect based only on the superior thyroid artery has not been reported previously. CASE REPORT: The case of a 40-year-old farmer with a large full-thickness cheek squamous cell carcinoma is reported. An SCM myocutaneous flap based on the SCM branch of the superior thyroid artery was used. CONCLUSION: An SCM myocutaneous flap is a valuable option for head and neck reconstructions with reasonable aesthetic results. For properly selected cases, this flap obviates the need to use the pectoralis major or other more distant flaps. LEVEL OF EVIDENCE V: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Evaluation of Sudden Sensory-Neural Hearing Loss Patients Treated with Systemic Steroids with Additional Intratympanic Dexamethasone Injection in Different Intervals; a Clinical Trial Study.

Sudden Sensory-Neural Hearing Loss (SSNHL) is one of the most important otologic emergency. Although adding intratympanic (IT) steroids to systematic steroid may be beneficial, the exact timing of the IT injections to provide the best response needs further investigations. To compare different protocols in treting sudden sensorineural hearing loss. We performed a clinical trial study on 120 patients from October 2021 to February 2022. All patients were prescribed 1 mg/Kg daily oral prednisolone. After randomization to three groups, the control group received standard twice a week IT steroid injections in 12 days (four total injections) while intervention groups 1 and 2 received once and twice a day IT injections for ten days. Audiometric study repeated 10-14 days after the last injection and assessed based on the Siegel criteria. We used the Chi-Square, Analysis of Variance (ANOVA), Kruskal-Wallis's tests where appropriate. The most clinical improvement was found in the standard treatment group, and group-2 had the greatest number of patients with no improvement; however, no overall significant difference was observed among the three groups (p-value: 0.066; Pearson Chi-Square). Less frequent IT injections in patients already on systemic steroids provide similar results to more frequent injections. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03641-4.

Frequency -Specific Air- Conduction and Bone - Conduction Outcomes after Stapedotomy.

Introduction: The aim of this study was to investigate hearing outcome of stapes surgery, considering the post-operative air and bone conduction (AC&BC) changes, in a frequency specific approach. Materials and Methods: This was a retrospective cohort study. A total of 245 ears (231 patients), who underwent Stapedotomy at our tertiary referral center in a period of 5 years were enrolled in the study. Pure tone audiometry (PTA) was evaluated preoperatively and one month postoperatively. AC, BC, and Air-bone gap (ABG) were documented. Moreover, one-year post-op PTA was also recorded for more than a quarter of the cases. Results: Overall, significant improvements were observed in AC thresholds with a mean AC gain of 20.44±13.64 dB. At higher frequencies the results were poorer (AC gain of 27 dB at 250 Hz vs 7 dB at 8000 Hz). ABG significantly improved at all frequencies after one month. BC thresholds were typically better after surgery. However, there appears to be a worsening trend in BC thresholds at frequencies higher than 2000 Hz. In 68 patients with 1-year follow-up, BC thresholds were slightly worse (but not statistically significant) at most frequencies, in comparison to the one-month results. Conclusions: Stapes surgery significantly improves air and bone conduction hearing, particularly at lower frequencies. Nonetheless, there exists a potential for sensorineural hearing loss (SNHL) at high frequencies. However, the changes are insignificant and not within the speech frequencies. Therefore, patients are typically satisfied with the hearing outcome of the surgery.

Complications and outcomes of cochlear implantation in children younger than 12 months: A multicenter study.

OBJECTIVES: Evidence suggests that Cochlear Implantation (CI) is a beneficial approach for auditory and speech skills improvement in children with severe to profound hearing loss. However, it remains controversial if implantation in children <12 months is safe and effective compared to older children. The present study aimed to determine whether children's ages affect surgical complications and auditory and speech development. METHODS: The current multicenter study enrolled 86 children who underwent CI surgery at <12 months of age (group A) and 362 children who underwent implantation between 12 and 24 months of age (group B). The Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were determined pre-impanation, and "one-year" and "two-year" post-implantation. RESULTS: All children had full insertions of the electrode array. Four complications (overall rate: 4.65%; three minor) occurred in group A and 12 complications (overall rate: 4.41%; nine minor) occurred in group B. We found no statistically significant difference in the complication rates between the groups (p > 0.05). The mean SIR and CAP scores improved over time following CI activation in both groups. However, we did not find significant differences in CAP and SIR scores between the groups across different time points. CONCLUSION: Cochlear implantation in children younger than 12 months is a safe and efficient procedure, providing substantial auditory and speech benefits. Furthermore, rates and nature of minor and major complications in infants are similar to those of children undergoing the CI at an older age.

Hepatitis C virus in patients with squamous cell carcinoma of the head and neck in Iran: is there any relation?

Recently, some studies have noticed a large number of patients with squamous cell carcinoma of the head and neck (SCCHN), who are infected with hepatitis C virus (HCV). The aim of this study is to determine the prevalence of HCV in these patients in our population. Patients with SCCHN in four tertiary centers in different regions of Iran were checked for HCV. The prevalence of HCV in 107 patients diagnosed with head and neck squamous cell carcinoma was 0.9%, which has no significant difference with its prevalence in normal population. It seems that HCV at least in Iran could not be considered as a risk factor for SCCHN.

Outcome of stapes surgery for tinnitus recovery in otosclerosis.

INTRODUCTION: Stapes surgery is mainly used to improve hearing in otosclerosis; however, many patients report alleviation of tinnitus after surgery. OBJECTIVE: to evaluate the effectiveness of stapes surgery for improving tinnitus symptoms. MATERIALS AND METHODS: This prospective study included 29 patients with surgically proven otosclerosis, who had undergone stapedectomy or stapedotomy. We compared symptomatic (tinnitus, hearing loss, and vertigo) and audiological findings obtained before and after surgery. Using Newman's method the level of discomfort caused by tinnitus was scored from grade I to V pre- and postoperatively. RESULTS: The mean preoperative tinnitus discomfort grade was 2.72, at day 1 after surgery it was 1.29, and after 1 month it had decreased to 0.96, indicating a significant improvement in the level of tinnitus discomfort (p < 0.0001). One month after surgery 82.8% of patients had a complete or partial absence of tinnitus. CONCLUSION: We conclude that stapes surgery is quite effective for treatment of tinnitus as well as improvement of hearing. When deciding upon management in patients with otosclerosis the presence of tinnitus should be considered as well as hearing level.

Comprehensive vestibular evaluation in patients with Otosclerosis: a case control study.

Aim: Otosclerosis is one of the common otologic diseases. The mechanism and the probable site of vestibular involvement are not yet fully understood. The present study aimed to perform a comprehensive vestibular evaluation in patients with otosclerosis, compared to the cases without otosclerosis. Materials and methods: patients underwent a comprehensive cochleovestibular evaluation, including audiometry, ocular and cervical vestibular evoked myogenic potential (o-VEMP and c-VEMP), video head impulse (vHIT) and caloric tests. The results were compared with those obtained from the non-otosclerosis control group. Results: A total of 61 individuals were included in the study who were divided into two groups of the case (50.82%) and control (49.18%). The results showed that there was a significant difference in the mean vHIT gain between the case and control groups (P < 0.05). However, the mean gain was still within the normal range. Besides, the patients with otosclerosis had significant bilateral or unilateral weaknesses according to caloric test results. Moreover, their o-VEMP and c-VEMP results were significantly abnormal as well (P < 0.05). Conclusion: Based on the results of the present study, the vestibular system even in asymptomatic cases, is affected by otosclerosis. Furthermore, it seems that the otolithic system has a higher chance of involvement, compared to the semicircular canals. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-022-03147-5.

Intratympanic Steroid for the Management of Sudden Hearing Loss: Introduction of a Tapering Method.

INTRODUCTION: Sudden sensorineural hearing loss (SSNHL) is a therapeutic challenge. There are several controversies regarding the management protocol of SSNHL. This study aimed to present the results of a novel treatment algorithm, which is a combination of systemic steroids and a tapering intratympanic (IT) dexamethasone regimen. MATERIALS AND METHODS: The past 10 years' medical records of idiopathic SSNHL cases in Ghaem and Emamreza hospital, Mashhad University of Medical Sciences were evaluated. Patients were assessed using standardized methods for pure-tone threshold audiometry. The management method of SSNHL treatment included oral steroids combined with IT administration of dexamethasone once a day for 7 days and continuing it on an alternate day and then weekly basis. Patients' recovery was assessed using Siegel's criteria. RESULTS: This study included a total of 248 cases of idiopathic sudden hearing loss, with a mean age of 40.63±16.19 years. In total, 105 (42.3%) and 143 (57.7%) patients were female and male, respectively. The most common associated symptoms included tinnitus (86.9%, n=205) followed by vertigo (52.1%, n=122). The final hearing outcome of patients showed that 39 (15.7%), 38(15.3%), 86 (34.7%), and 85 (34.3%) patients underwent a complete recovery, partial recovery, slight recovery, and no recovery, based on Siegel's criteria. CONCLUSIONS: The dose, interval, and duration of IT steroid treatment were not universally approved. The treatment method designed based on a tapering of IT steroid injection in combination with already known systemic administration of steroids can be a treatment option in SSNHL patients.

Hyperbilirobinemia and Its Effect on Auditory Perception and Speech Intelligibility in Cochlear Implanted Children (Cochlear Implantation Outcomes in Children Who Have Experienced Hyperbilirobinemia).

The goal of this study was to investigate the probable difference in auditory perception and speech intelligibility performance amongst cochlear implanted children who experienced hyperbilirubinemia or auditory neuropathy in comparison to the cochlear implanted children with unknown etiology for hearing loss. This case-control study was carried out on 106 cochlear implanted children with mean age of 32.36 ± 11.98 months who were purposively selected and allocated into four groups. Out of the total, 30 had no specific etiology for hearing loss, while the others had experienced auditory neuropathy or hyperbilirubinemia with/without blood exchange. The auditory perception and speech intelligibility performance of all the participants who had received auditory verbal therapy were assessed after 6 and 12 months of rehabilitation. Then, the data was analyzed, using the Statistical Package for Social Sciences-version 21(SPSS-21). Results indicated poor auditory perception and speech intelligibility performance of the cochlear implanted children with hyperbilirubinemia and blood exchange (P ≤ 0.05), while the participants in the control group with no specific etiology for hearing loss, the children with hyperbilirubinemia with no blood exchange, and those who suffered from auditory neuropathy performed better, respectively. Also, a significant correlation between auditory neuropathy and hyperbilirubinemia was observed. Despite lower improvement of auditory perception and speech intelligibility of the hearing impaired children who were experiencing moderate to severe degrees of hyperbilirubinemia or auditory neuropathy, cochlear implantation is highly recommended not only for children with unknown etiology for severe hearing loss but also for this group of hearing impaired children.

Association Between Serum Vitamin D Level and Ménière's Disease.

OBJECTIVES: Ménière's disease (MD) is a well-known inner ear disease; however, the etiopathogenesis is unknown. Several factors may be involved. Meanwhile, vitamin D is reported to have an important role in inner ear physiology. The aim of this study is to evaluate the relation between vitamin D deficiency and MD. STUDY DESIGN: This matched case-control study compared serum vitamin D levels between patients with definite MD and those without it. SETTING: The study was done between August 2018 and December 2019 at Ghaem University Hospital in Mashhad, Iran. METHODS: Twenty-eight patients with definite MD were matched with a group of 84 healthy individuals, regarding age, sex, body mass index, and occupation (indoor vs outdoor). The serum level of vitamin D (25-hydroxyvitamin D3) was measured in both groups. RESULTS: The mean ± SD vitamin D level was 18.9 ± 9.7 ng/mL in the case group and 25.2 ± 13.7 ng/mL in the control group (P = .027). There was a significant difference between the case and control groups according to the results of the conditional logistic regression model (P = .03; adjusted odds ratio, 0.96). In the MD group, 17 (60.7%) patients were vitamin D deficient, 6 (21.4%) insufficient, and only 5 (17.9%) sufficient. CONCLUSIONS: The results of this study show that serum vitamin D level in MD is significantly lower than that of the control group. However, the role of vitamin D supplementation in the management of MD needs further study. LEVEL OF EVIDENCE: 4.

Identification of three novel homozygous variants in COL9A3 causing autosomal recessive Stickler syndrome.

BACKGROUND: Stickler syndrome (STL) is a rare, clinically and molecularly heterogeneous connective tissue disorder. Pathogenic variants occurring in a variety of genes cause STL, mainly inherited in an autosomal dominant fashion. Autosomal recessive STL is ultra-rare with only four families with biallelic COL9A3 variants reported to date. RESULTS: Here, we report three unrelated families clinically diagnosed with STL carrying different novel biallelic loss of function variants in COL9A3. Further, we have collected COL9A3 genotype-phenotype associations from the literature. CONCLUSION: Our report substantially expands the molecular genetics and clinical basis of autosomal recessive STL and provides an overview about allelic COL9A3 disorders.

The Outcome of Cochlear Implantations in Deaf-Blind Patients: A Multicenter Observational Study.

OBJECTIVE: This multicenter study aimed to evaluate the auditory and speech outcomes of cochlear implantation (CI) in deaf-blind patients compared with deaf-only patients. STUDY DESIGN: Retrospective cohort study. SETTING: Multiple cochlear implant centers. PATIENTS: The current study was conducted on 17 prelingual deaf-blind children and 12 postlingual deaf-blind adults who underwent CI surgery. As a control group, 17 prelingual deaf children and 12 postlingual deaf adults were selected. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Auditory and linguistic performances in children were assessed using the categories of auditory performance (CAP) and Speech Intelligibility Rating (SIR) scales, respectively. The word recognition score (WRS) was also used to measure speech perception ability in adults. The mean CAP, SIR, and WRS cores were compared between the deaf-only and deaf-blind groups before CI surgery and at "12 months" and "24 months" after device activation. Cohen's d was used for effect size estimation. RESULTS: We found no significant differences in the mean CAP and SIR scores between the deaf-blind and deaf-only children before the CI surgery. For both groups, SIR and CAP scores improved with increasing time after the device activation. The mean CAP scores in the deaf-only children were either equivalent or slightly higher than those of the deaf-blind children at "12 months post-CI" (3.94 ± 0.74 vs 3.24 ± 1.25; mean difference score, 0.706) and "24 months post-CI" (6.01 ± 0.79 vs 5.47 ± 1.06; mean difference score, 0.529) time intervals, but these differences were not statistically significant. The SIR scores in deaf-only implanted children were, on average, 0.870 scores greater than the deaf-blind children at "12 months post-CI" (2.94 ± 0.55 vs 2.07 ± 1.4; p = 0.01, d = 0.97) and, on average, 1.067 scores greater than deaf-blind children at "24 months post-CI" (4.35 ± 0.49 vs 3.29 ± 1.20; p = 0.002; d = 1.15) time intervals. We also found an improvement in WRS scores from the "preimplantation" to the "12-month post-CI" and "24-month post-CI" time intervals in both groups. Pairwise comparisons indicated that the mean WRS in the deaf-only adults was, on average, 10.61% better than deaf-blind implanted adults at "12 months post-CI" (62.33 ± 9.09% vs 51.71 ± 10.73%, p = 0.034, d = 1.06) and, on average, 15.81% better than deaf-blind adults at "24-months post-CI" (72.67 ± 8.66% vs 56.8 ± 9.78%, p = 0.002, d = 1.61) follow-ups. CONCLUSION: Cochlear implantation is a beneficial method for the rehabilitation of deaf-blind patients. Both deaf-blind and deaf-only implanted children revealed similar auditory performances. However, speech perception ability in deaf-blind patients was slightly lower than the deaf-only patients in both children and adults.