Thermal Analysis of an Ultrasonic Aspirator Micro Claw Tool Compared With Standard High-Speed Drilling During Internal Auditory Canal Opening in a Cadaveric Model.

BACKGROUND: The ultrasonic aspirator micro claw tool (UAmCT) can be used to remove the bone of the internal auditory canal (IAC) during vestibular schwannoma resection via the retrosigmoid approach (RSA) without the risk of a spinning drill shaft. However, the thermal profile of the UAmCT during IAC removal has not been reported. OBJECTIVE: To compare the thermal profile of the UAmCT during access of the IAC to that of a conventional high-speed drill (HSD) and to present an illustrative case of this application. METHODS: IAC opening via RSA was performed in 5 embalmed cadaveric specimens using the UAmCT with 3, 8, and 15 mL/min irrigation on the left and the HSD at 75 000 revolutions per minute and 0%, 14%, and 22% irrigation on the right. Peak bone surface temperatures were measured 4 times in 20-second intervals, and statistical analyses were performed using SPSS software. An illustrative case of a vestibular schwannoma resected via an RSA using the UAmCT to access the IAC is presented. RESULTS: The IAC was opened in all 5 specimens using both the UAmCT and HSD without complication. The mean peak bone surface temperatures were significantly lower with the UAmCT compared with the HSD ( P < .001). The UAmCT did not meaningfully prolong the operating time in the illustrative case, and the IAC was accessed without complication. CONCLUSION: The UAmCT may be a safe and effective alternative to HSD for IAC opening during vestibular schwannoma resection via the RSA. Larger studies under clinical conditions are required to further validate these findings.

Otolaryngologic manifestations of Mpox: the Atlanta outbreak.

BACKGROUND: In 2022, Mpox (MPX) has become clinically relevant as otolaryngologists are evaluating this exotic disease process due to its many otolaryngologic manifestations. AIMS/OBJECTIVE: To characterize our cohort of otolaryngology-relevant MPX confirmed cases. MATERIALS AND METHODS: A descriptive case series was performed via retrospective review. Adult patients who underwent inpatient or emergency department otolaryngology consultation at an Emory University-affiliated tertiary care level hospital for MPX were included. RESULTS: Seven patients (age 18-58 years; median 32 years) were identified. All patients were male. Six patients (86%) were black and six patients (86%) were HIV positive with varied immunocompetence. Otolaryngology was consulted for lymphadenopathy (n = 2), pharyngeal involvement (n = 1), and airway evaluation (n = 4). All 6 patients with active MPX developed the classic rash, which developed after oropharyngeal symptoms in 3 patients. Three patients had laryngeal involvement. CONCLUSION: MPX manifests with symptoms requiring otolaryngology expertise, especially when the airway is involved. Infectious disease consultation is key. Mpox can be identified with a specific constellation of demographic identifiers and physical exam findings, which is key to determining appropriate treatment and protection for the consulting otolaryngologist. SIGNIFICANCE: This is the first otolaryngologic study of Mpox and first description of Mpox laryngeal involvement.

Surgical management of spontaneous middle cranial fossa defects: a systematic review and meta-analysis of available reconstructive techniques and materials.

Different materials and techniques have been proposed for surgical repair of spontaneous middle cranial fossa (MCF) defects. However, conclusive evidence supporting their selection and impact on clinical outcomes is lacking. The study aims to conduct a systematic review and meta-analysis on materials and techniques employed to repair MCF defects and evaluate complications and rates of recurrent cerebrospinal fluid (CSF) leaks. A PRISMA-guided systematic review and meta-analysis were performed using MESH terms and specific keywords including studies published before May 2022. Primary outcomes included recurrence of CSF leak and complication rates by type of reconstructive material and technique utilized. Meta-analyses of proportions were performed using random effects and confidence intervals for individual proportions were calculated using the Clopper-Pearson method. Twenty-nine studies were included (n = 471 cases). Materials employed for repair were categorized according to defect size: 65% of defects were of unknown size, 24% were small (< 1 cm), and 11% were large (≥ 1 cm). Rigid reconstruction (RR) was significantly favored over soft reconstruction (SR) for larger defects (94% of cases, p < 0.05). Complications and recurrent CSF leak rates of SR and RR techniques were comparable for defects of all sizes (p > 0.05). Complication rates reported for these procedures are low regardless of technique and material. RR was universally preferred for larger defects and analysis of complication and recurrence rates did not reveal differences regardless of defect size. While RR was more frequently reported in smaller defects, SR was used by several centers, particularly for smaller MCF floor defects.

Contralateral Transmaxillary Approach to Petrous Apex Granuloma with Lateral Maxillotomy: 2-Dimensional Operative Video.

The endoscopic endonasal approach (EEA) has proven to be a valuable tool to access the petrous apex (PA).1 Although lesions located medial to the PA can be easily accessed through an EEA with minimal risk to the internal carotid artery (ICA), more laterally seated lesions represent a surgical challenge. The contralateral transmaxillary approach is an effective extension of the normal EEA to the PA, allowing a more lateral trajectory and thereby reducing the risks connected to paraclival ICA manipulation.2-4 This is a 47-year-old female presenting with tinnitus, hearing loss, and right facial twitching. The preoperative audiogram showed a 60 dB sensorineural hearing loss, and magnetic resonance imaging demonstrated an expansive lesion of the PA with high signal intensity in T1- and T2-weighted sequences. A high-riding jugular bulb ruled out the use of an infracochlear infralabyrinthine approach, and a middle cranial fossa approach was felt to render a high risk of recurrence due to the potential for incomplete resection. Ultimately, a contralateral transmaxillary approach was selected. Due to the extreme lateral position of the lesion, the maxillotomy was created in the lateral wall of the maxillary sinus to allow a coaxial approach to the PA. Postoperatively, the patient did not report new neurologic deficits. Anatomic pathology was consistent with cholesterol granuloma (Video 1). In the surgical management of PA granulomas, recognizing relevant anatomic structures such as the paraclival ICA, cochlea, jugular bulb, and vestibule preoperatively is of paramount importance to tailor the surgical approach and reduce its risks.

Exoscope-Assisted Middle Cranial Fossa Approach for Repair of Tegmental Defects: A Cadaveric and Clinical Study.

BACKGROUND: Recent studies highlighted how exoscopes may be employed to approach the lateral skull base. The use of exoscope-assisted procedures to repair middle cranial fossa (MCF) defects has not been fully explored. The surgical microscope in the same circumstances has been associated with relevant limitations, such as its physical obstruction, among others. The aim of this study was to present a proof of concept of exoscope-assisted surgery for MCF defects. METHODS: A detailed step-by-step MCF approach was performed on 2 alcohol-preserved, latex-injected cadaveric specimens under exoscopic magnification. An illustrative clinical case of encephalocele secondary to a spontaneous tegmen tympani defect repaired via an exoscope-assisted MCF approach was presented. RESULTS: The most common sites of MCF defects, the tegmen tympani and the arcuate eminence, were successfully exposed under exoscopic magnification. Dissection was easily performed; no damage to the dura mater or to vascular or neural structures occurred. In the clinical case, the exoscope-assisted technique demonstrated adequate maneuverability and magnification quality. After localization, the encephalocele was resected, and the MCF defect was repaired. The surgeon's position was comfortable, and operative time was not prolonged. CONCLUSIONS: The exoscope allows adequate exposure of the MCF floor with identification and preservation of key anatomical structures. The exoscope represents a valuable alternative to the microscope in reconstruction of MCF defects, offering high-quality magnification and proven maneuverability.

Retrosigmoid Vestibular Neurectomy for Meniere Disease: A Technical Note.

BACKGROUND: Meniere disease (MD) is an idiopathic peripheral pathology involving the acoustic apparatus. One of the most critical challenges in managing MD is intractable vertigo. In this context, retrosigmoid vestibular neurectomy has been described as a safe and effective technique to manage this symptom when it is resistant to first- and second-line treatments. This article analyzed the alternative treatment options, specific surgical anatomy, and relevant details of vestibular neurectomies performed for intractable MD. METHODS: Relevant neurovascular landmarks, critical surgical steps, adequate indications, and potential pitfalls of retrosigmoid vestibular neurectomy were analyzed based on an illustrative clinical case of intractable MD. RESULTS: The illustrative case demonstrated how early recognition of the facial nerve and the vestibulocochlear plane is fundamental to performing retrosigmoid vestibular neurectomy. This procedure is indicated in cases of resistant MD with preoperative hearing integrity. Potential pitfalls of this technique are incomplete neurotomy, nerve regeneration, comorbidities in the contralateral ear, adverse anatomy, the possibility of nonotologic vertigo, and incomplete vestibular compensation. CONCLUSIONS: Vestibular neurectomy represents a safe and effective technique to manage MD that is resistant to medical treatment, allowing symptom control and hearing preservation. Nevertheless, detailed knowledge of surgical anatomy and possible pitfalls is of paramount importance to achieve a good outcome.

Racial Disparities in Adult Cochlear Implantation.

OBJECTIVE: To compare rates of cochlear implant referral and cochlear implantation across different races and to compare audiometric profiles of these patients. STUDY DESIGN: Retrospective study. SETTING: Academic tertiary care institution. METHODS: Demographic and audiometric data were collected for patients who underwent cochlear implant evaluation or cochlear implantation from 2010 to 2020. RESULTS: A total of 504 patients underwent cochlear implant evaluation; 388 met cochlear implant candidacy criteria, and 258 underwent implantation. Of the patients referred for cochlear implant evaluation, 68.5% were White, 18.5% were Black, and 12.3% were Asian, while the institution's primary service area is 46.9% White, 42.3% Black, and 7.7% Asian (P < .001). Black patients referred for cochlear implant evaluation had significantly worse hearing (mean pure-tone average [PTA] 84.5 dB, 26.1% word recognition) than White patients (mean PTA 78.2 dB, P = .005; 35.7% word recognition, P = .015) and Asians patients (mean PTA 77.9 dB, P = .04; 36.5% word recognition, P = .04). Black patients who underwent cochlear implant evaluation also had significantly worse AzBio scores than White patients: 24.5% versus 36.7% (P = .003). There was no significant difference in cochlear implantation rates between Black and White candidates (P = .06). CONCLUSION: Black patients undergo cochlear implant evaluation and cochlear implantation at rates disproportionately lower than expected based on local demographics. In addition, Black patients have significantly worse hearing at the time of cochlear implant referral than White and Asian patients. Identifying and increasing awareness of these disparities are essential steps to improving cochlear implant access for potentially disadvantaged populations.

Adherence to Long-Term Follow-up in Patients With Sporadic Vestibular Schwannomas Managed With Serial Observation.

OBJECTIVES: To examine the long-term adherence to serial imaging of patients with sporadic vestibular schwannoma and analyze factors associated with being lost to follow-up. STUDY DESIGN: Retrospective chart review with telephone interview. SETTING: Single tertiary care center. METHODS: Patients with a sporadic vestibular schwannoma and started on observational surveillance management between January 2005 and December 2010 were included. Demographic data, tumor size, hearing and vestibular changes, and follow-up length were recorded. Patient factors were analyzed for association with being lost to follow-up. RESULTS: In total, 122 patients were included with a median length of follow-up of 5 months (range, 0-146). After initial surveillance, 22.1% (n = 27) of patients had a change in management to either microsurgery or radiosurgery. Of the remaining 77.9% (n = 95), nearly half (44.2%, n = 42) never returned for a second visit, and all but 3 were eventually lost to follow-up. There was no association between sex, race, age at diagnosis, initial tumor size, insurance status, household income, or driving distance to hospital and being lost to follow-up. Of 26 interviewed patients initially lost to follow-up, 11 (42.3%) sought care at another institution, 5 (19.2%) chose to no longer receive care, 1 (3.8%) had transportation difficulties, and 9 (36.4%) had poor understanding of their diagnosis or instructions. CONCLUSIONS: The length of follow-up for patients undergoing surveillance of sporadic vestibular schwannoma varies widely, and patients are commonly lost to follow-up. Further efforts should be made to identify at-risk patients and provide adequate education to improve long-term surveillance.

Unilateral Facial Paralysis in the Pediatric Patient.

Unilateral facial paralysis (FP) in the pediatric population is a rare entity secondary to multiple etiologies including infectious, vascular, and neoplastic. In persistent or recurrent FP, imaging can demonstrate a peripheral facial nerve (FN) lesion. Given the rarity of FN lesions, however, there is limited literature regarding optimal management. In this case series, we describe the presentation, evaluation, and management of unilateral FP in three pediatric patients along with a review of the literature. All patients presented with complete FP due to a peripheral FN lesion or compression of the FN. A combined mastoid and middle cranial fossa approach was utilized for excision in two cases, and the other child underwent a translabyrinthine approach. The pathology of the lesions revealed a meningioma, an arachnoid cyst, and a hemangioma. Presentation, evaluation, post-operative outcomes, as well as final pathologies are discussed.

Spontaneous Skull Base Cerebrospinal Fluid Leaks and Their Relationship to Idiopathic Intracranial Hypertension.

BACKGROUND: The association between spontaneous skull base cerebrospinal fluid (CSF) leaks and idiopathic intracranial hypertension (IIH) has been suggested, but its significance remains unclear. OBJECTIVE: To estimate the prevalence of IIH in spontaneous skull base CSF leak patients. METHODS: Systematic collection of demographics, neuro-ophthalmic and magnetic resonance imaging evaluation of spontaneous skull base CSF leak patients seen pre- and post-leak repair in one neuro-ophthalmology service. Patients with preexisting IIH were diagnosed with definite IIH if adequate documentation was provided; otherwise, they were categorized with presumed IIH. Classic radiographic signs of intracranial hypertension and bilateral transverse venous sinus stenosis were recorded. RESULTS: Thirty six patients were included (age [interquartile range]: 50 [45;54] years; 94% women; body mass index: 36.8 [30.5;39.9] kg/m2), among whom six (16.7%, [95% confidence interval, CI]: [6.4;32.8]) had a preexisting diagnosis of definite or presumed IIH. Of the remaining 30 patients, four (13.3%, 95%CI: [3.8;30.7]) had optic nerve head changes suggesting previously undiagnosed IIH, while one was newly diagnosed with definite IIH at initial consultation. One out of 29 patients with normal findings of the optic nerve head at presentation developed new onset papilledema following surgery (3.4%, 95%CI: [0.1;17.8]) and was ultimately diagnosed with definite IIH. Overall, the prevalence of definite IIH was 19.4% (95%CI: [8.2;36.0]). CONCLUSION: Striking demographic overlap exists between IIH patients and those with spontaneous CSF leak. Definite IIH was present in approximately 20% of our patients. However, its true prevalence is likely higher than identified by using classic criteria. We therefore hypothesize that an active CSF leak serves as an auto-diversion for CSF, thereby "treating" the intracranial hypertension and eliminating characteristic signs and symptoms at initial presentation.

Comprehensive genetic testing of Chinese SNHL patients and variants interpretation using ACMG guidelines and ethnically matched normal controls.

Hereditary hearing loss is a monogenic disease with high genetic heterogeneity. Variants in more than 100 deafness genes underlie the basis of its pathogenesis. The aim of this study was to assess the ratio of SNVs in known deafness genes contributing to the etiology of both sporadic and familial sensorineural hearing loss patients from China. DNA samples from 1127 individuals, including normal hearing controls (n = 616), sporadic SNHL patients (n = 433), and deaf individuals (n = 78) from 30 hearing loss pedigrees were collected. The NGS tests included analysis of sequence alterations in 129 genes. The variants were interpreted according to the ACMG/AMP guidelines for genetic hearing loss combined with NGS data from 616 ethnically matched normal hearing adult controls. We identified a positive molecular diagnosis in 226 patients with sporadic SNHL (52.19%) and in patients from 17 deafness pedigrees (56.67%). Ethnically matched MAF filtering reduced the variants of unknown significance by 8.7%, from 6216 to 5675. Some complexities that may restrict causative variant identification are discussed. This report highlight the clinical utility of NGS panels identifying disease-causing variants for the diagnosis of hearing loss and underlines the importance of a broad data of control and ACMG/AMP standards for accurate clinical delineation of VUS variants.

The Prevalence of Superior Semicircular Canal Dehiscence in Patients With Mastoid Encephalocele or Cerebrospinal Fluid Otorrhea.

OBJECTIVE: To evaluate the association between mastoid encephalocele or cerebrospinal fluid (CSF) otorrhea and concurrent superior semicircular canal dehiscence (SSCD). STUDY DESIGN: Retrospective case-control study with chart and imaging review. SETTING: University-affiliated tertiary referral center. PATIENTS: A chart review was conducted of all patients greater than 18 years of age who had primary surgery for CSF otorrhea or encephalocele at our university-affiliated tertiary center from 2000 to 2016. Eighty-three patients matched inclusion criteria for case subjects, and 100 patients without CSF otorrhea or encephalocele were included for controls. MAIN OUTCOME MEASURE: High-resolution computed tomography (CT) scans were reviewed to assess superior semicircular canal roof integrity. Student's t test was used to determine significance of continuous variables. Odds ratio (OR) and χ test was used to determine the association between SSCD and concurrent mastoid encephalocele or CSF otorrhea compared with the control population. RESULTS: The mean age of the 83 case subjects was 54 years, and 73% were women. In patients with confirmed encephalocele and CSF otorrhea, the prevalence of SSCD was 35%, which was significantly greater than controls (2%) (OR = 26.1, p < 0.001). In patients with only CSF otorrhea, 21% had concurrent SSCD (OR = 10.3, p = 0.001). In patients with SSCD, 46% had bilateral canal dehiscence. CONCLUSIONS: This is the largest study to evaluate the prevalence of SSCD in patients with concurrent mastoid encephalocele. This study showed a significant association between SSCD and the presence of both mastoid encephalocele and CSF otorrhea. This data suggests that surgeons should have a high suspicion for SSCD in patients presenting with encephalocele or CSF otorrhea.

Landmarks for the preservation of the middle temporal artery during mastoid surgery: Cadaveric dissection study.

IMPORTANCE: The middle temporal artery flap is a vascularized periosteal flap that is highly useful for otologic reconstruction including the middle cranial fossa, mastoidectomy defect, and external auditory canal. The course of the artery is close to the external auditory canal and is easily injured during preliminary exposure and elevation of flaps. OBJECTIVES: To describe the course of the middle temporal artery in relation to the external auditory canal and the superficial temporal artery in order to enhance preservation and use in otologic reconstruction. DESIGN: Dissection of preserved, injected cadaveric temporal bones. SETTING: Anatomical laboratory. PARTICIPANTS: Seven cadaveric temporal bones. INTERVENTION: Temporal bones were dissected in a planar manner to identify the middle temporal artery along the squamous temporal bone to its origin. The superior border of the external auditory canal was divided, horizontally, into thirds to create three measurement points. Distances between the middle temporal artery and the bony portion of the external auditory canal were then determined. MAIN OUTCOMES AND MEASURES: Horizontal diameter of the external auditory canal, distance from the superior-most border of the external auditory canal to the middle temporal artery, various patterns of the middle temporal artery. RESULTS: The middle temporal artery branched from the superficial temporal artery in all specimens. Mean horizontal diameter of the external auditory canal was 9.97mm. Mean distances between the bony portion of the external auditory canal and middle temporal artery for the first, second, and third points along the horizontal diameter of the external auditory canal were 1.57, 2.96, and 4.02mm, respectively. In at least one specimen, the artery dipped into the external auditory canal. CONCLUSIONS AND RELEVANCE: The middle temporal artery runs closest to the external auditory canal at the anterosuperior border. To preserve the middle temporal artery for use in reconstruction after otologic surgery, the surgeon should avoid dissection superior to the external auditory canal until the artery is positively identified.

Review of a single surgeon's stapedotomy cases performed with a nickel titanium prosthesis over a 14-year period.

CONCLUSION: Stapes surgery with a nickel titanium prosthesis is a safe and well-tolerated procedure that leads to a significant improvement in hearing outcomes. OBJECTIVE: To identify the efficacy and safety of stapedotomy procedures performed with a nickel titanium prosthesis for patients with otosclerosis. METHODS: A review of 431 unique stapedotomies performed over 14 years by a single surgeon at an academic tertiary care center yielded 312 cases with nickel titanium prosthesis that met inclusion criteria of otosclerosis diagnosis, initial surgery in operative ear, and presence of pre-operative and post-operative audiograms. Pure-tone averages (PTA) at baseline and 8 weeks after surgery were calculated over four frequencies; 0.5, 1, 2, and 4 kHz. Average air-bone gaps (ABG) were calculated from pre-operative and post-operative audiograms. RESULTS: Average pre-operative baseline PTA was 56.7 dB in the affected ear. Post-operative PTA was 30.1 dB, a 26.6 dB improvement. Initial average ABG was 29.7 dB, while post-operative ABG averaged 5.4 dB, a 24.2 dB improvement. Surgical success (closure of ABG within 10 dB) was achieved in 263 (84%) patients. Rate of surgical success was not correlated with age, gender, race, or affected ear. Complications included recurrent conductive hearing loss (14), progressive SNHL (4), and post-operative BPPV (3).

Diagnostic Challenges in a Case of IgG4-RD Affecting the Temporal Bone.

INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is a recently described fibroinflammatory condition with a characteristic histology. While IgG4-RD can affect a great variety of anatomical sites, it has been seldom described in the temporal bone. METHODS: Herein, a case IgG4-RD occurring in the temporal bone of a 35-year-old woman is reported. DISCUSSION: This case of IgG4-RD of the temporal bone proved a uniquely challenging diagnosis due to slightly atypical histology falling outside of "highly suggestive" criteria. CONCLUSIONS: We suggest that IgG4-RD remains a challenging diagnosis to reach despite increased awareness of the condition. We further suggest that clinicopathologic correlation remain the cornerstone of diagnosis as the spectrum of presentations of this newly described disease may be wider than previously anticipated.

Spontaneous defects between the mastoid and posterior cranial fossa.

Conclusions Spontaneous defects between the mastoid and the posterior cranial fossa are exceedingly rare. Patients with these lesions may have a lower BMI compared to those with middle cranial fossa encephaloceles, but are otherwise demographically similar. This study recommends repair via a transtemporal approach to allow for examination of the entire posterior face of the temporal bone. Objective To describe cases of spontaneous posterior cranial fossa defects. Methods This study reviewed all cases of spontaneous posterior fossa defects presenting to a tertiary referral center over the last decade and described clinical presentation, imaging, operative findings, and outcomes. We also compared these lesions to those previously reported in the literature as well as the more common spontaneous encephaloceles of the middle cranial fossa. Results This study identified five cases with a mean age of 61.4 years, female-to-male ratio of 4:1, and a mean BMI of 31. Three cases presented with spontaneous pneumocephalus, one with CSF otorrhea, and one as an incidental imaging finding. Four defects were found medial to the sigmoid sinus and one was in the lateral retrosigmoid air cells.

Cerebrospinal fluid leaks following septoplasty.

We conducted a retrospective review to identify the characteristics of cerebrospinal fluid (CSF) leak in patients who had undergone septoplasty and in selected patients who had experienced a spontaneous CSF leak. CSF leak is a known but infrequently reported complication of septoplasty; to the best of our knowledge, only 4 cases have been previously published in the literature. A review of our institution's database revealed 3 cases of postseptoplasty CSF leak. We reviewed all the available data to look for any commonalities among these 7 cases. In addition, we reviewed 6 cases of spontaneous CSF leak selected from our database for the same purpose. For all patients, we noted the side of the cribriform plate defect, its size and, for the postseptoplasty cases, the interval between the septoplasty and the leak repair. Overall, we found that leaks were much more common on the right side than on the left. The sizes of the leaks in the 2 postseptoplasty groups were comparable (mean: 14.0 × 6.4 mm). The interval between septoplasty and leak repair ranged from 2.5 to 20 years in our cases and from 3 days to 22 weeks in the previously published cases. All 3 of the postseptoplasty patients in our database presented with clear rhinorrhea. Two of the 3 patients had meningitis; 1 of these 2 also had pneumocephalus. Of the 6 cases of spontaneous CSF leaks, 4 occurred on the right and 2 on the left; the average size of the defect was 5.8 mm in the greatest dimension. The finding that cribriform plate defects after septoplasty were typically right-sided likely reflects the prevalence of left-sided surgical approaches. Also, the fact that the defects were larger in the postseptoplasty cases than in the spontaneous cases is likely attributable to the torque effect toward the thin skull base that occurs when the perpendicular plate is twisted during septoplasty.

A low-cost exon capture method suitable for large-scale screening of genetic deafness by the massively-parallel sequencing approach.

Current major barriers for using next-generation sequencing (NGS) technologies in genetic mutation screening on an epidemiological scale appear to be the high accuracy demanded by clinical applications and high per-sample cost. How to achieve high efficiency in enriching targeted disease genes while keeping a low cost/sample is a key technical hurdle to overcome. We validated a cDNA-probe-based approach for capturing exons of a group of genes known to cause deafness. Polymerase chain reaction amplicons were made from cDNA clones of the targeted genes and used as bait probes in hybridization for capturing human genomic DNA (gDNA) fragments. The cDNA library containing the clones of targeted genes provided a readily available, low-cost, and regenerable source for producing capture probes with standard molecular biology equipment. Captured gDNA fragments by our method were sequenced by the Illumina NGS platform. Results demonstrated that targeted exons captured by our approach achieved specificity, multiplexicity, uniformity, and depth of coverage suitable for accurate sequencing applications by the NGS systems. Reliable genotype calls for various homozygous and heterozygous mutations were achieved. The results were confirmed independently by conventional Sanger sequencing. The method validated here could be readily expanded to include all-known deafness genes for applications such as genetic hearing screening in newborns. The high coverage depth and cost benefits of the cDNA-probe-based exon capture approach may also facilitate widespread applications in clinical practices beyond screening mutations in deafness genes.