A new phenomenon of cochlear otosclerosis: an acquired or congenital disease? - A clinical report of cochlear otosclerosis.

BACKGROUND: No cochlear otosclerosis in infants with congenital bilateral SNHL has been reported. AIMS/OBJECTIVES: We report an infant male with bilateral cochlear otosclerosis, suggesting that cochlear otosclerosis may be a congenital disease and to further analyze the etiology of and genetic expression in congenital bilateral cochlear otosclerosis. We also describe the clinical characteristics and experience of patients with bilateral cochlear otosclerosis treated with cochlear implants (CIs). MATERIALS AND METHODS: Seven patients, including an infant, who were diagnosed with cochlear otosclerosis underwent CI surgery. Their medical records, audiological and radiological results, surgical procedures, and CI outcomes were collected and reviewed. RESULTS: The median age at hearing loss was 38 years, ranging from 0 to 47 years. The child had bilateral hearing loss at birth and received a CI at 1 year of age. He also had growth retardation and was diagnosed with 3q+/3p- syndrome. All patients (8 ears) had better postoperative auditory performance than that preoperatively. CONCLUSIONS AND SIGNIFICANCE: Although cochlear otosclerosis often starts at middle age and progresses slowly, it may be a congenital disease that is related to chromosome abnormality. This disease presents with SNHL or MHL, and treatment with a CI is beneficial.

Case study: cochlear implantation in cochlear otospongiosis.

Objective and importance: Cochlear implantation can be performed successfully in patients with otospongiosis of the temporal bone with the potential for excellent audiological outcomes and high patient satisfaction. The purpose of this case report is to highlight the clinical considerations for implantation in cochlear otospongiosis including the need for careful pre-operative implant device selection, intra-operative surgical challenges such as the presence of hypervascularity and possible cochlear ossification resulting in difficulty in placing the electrode array and the possibility of postoperative facial nerve stimulation.Clinical presentation: A 14-year-old girl with cochlear otospongiosis likely due to osteogenesis imperfecta presented with progressive bilateral profound sensorineural hearing loss underwent successful cochlear implantation despite several challenges.Conclusion: Cochlear implantation in patients with cochlear otospongiosis with profound sensorineural hearing loss potentially may be very successful. Thorough pre-operative radiological evaluation is necessary. Possible intra-operative and post-operative challenges unique to these patients must be kept in mind. Adequate precautions should be taken to optimize the likelihood of complete electrode insertion such as using a depth gauge prior to inserting the electrode array and performing an intra-operative x-ray and / or neural response telemetry to confirm correct electrode placement.

[A case of congenital (juvenile) otosclerosis].

The present paper reports a case of congenital (juvenile) otosclerosis. This pathology is known to occur in 10% of the patients and cause severe hearing impairment. Precise diagnostics of the activity and extent of the otosclerotic process based on the results of functional multispiral computed tomography (MSCT) enables the practicing surgeons to identify indications for the surgical intervention, choose the adequate method and time for its performance, and make prognosis of its outcome. This inference is confirmed by the clinical observation described in this communication.

Hearing results of stapedotomy and malleo-vestibulopexy in congenital hearing loss.

AIMS: To analyze hearing results of surgical treatment of hearing loss associated with the congenital stapes ankylosis with or without malformations of ossicular chain. STUDY DESIGN: Retrospective chart review. METHODS: The charts of 1369 stapedotomies performed by senior author (JH) from 1991 to 2006 were reviewed. In 40 cases operative findings were consistent with isolated congenital stapes fixation or associated with middle ear malformations. The modified stapedotomy technique was employed in 33 cases and malleo-vestibulopexy was used in 7 cases. Operative findings were standardized according to Cremers' classification. The outcomes of 40 surgeries were analyzed according to the 1995 AAO-HNS Committee on Hearing and Equilibrium guidelines. High frequency hearing results on 4, 8 and 12kHz were reported in addition to standard frequencies. Results of stapedotomies and malleo-vestibulopexies were calculated separately. Surgical complications were described. RESULTS: The mean post-operative air conduction (AC) was 33 dB, bone conduction (BC) 22 dB and speech reception thresholds (SRT) 31 dB. Closure of the air-bone gap (ABG) to within 10 dB was achieved in 24/40 (60%) of cases. Lack of improvement was observed in 3/40 (8%) patients. In 26/32 (81%) of cases with potential for bilaterally serviceable hearing it was achieved. In 24/40 (60%) of cases symmetrical hearing with interaural difference of less than 10 dB was demonstrated. CONCLUSION: Significant hearing gain in patients with congenital stapes ankylosis makes surgical treatment a valuable adjunct or an alternative to hearing aids in selected cases.

Stapedectomy in congenital stapes fixation: are hearing outcomes poorer?

OBJECTIVE: To evaluate outcomes of stapedectomy surgery for congenital stapes fixation. STUDY DESIGN: Retrospective chart review. METHODS: The charts of 463 stapedectomies performed by 1 surgeon from 1996 to 2003 were reviewed. Patients with a history of childhood hearing loss and operative findings consistent with congenital fixation of the stapes were included. Patients with a history of trauma or chronic otitis media and those with otosclerosis were excluded. Thirty-six patients underwent stapedectomy for congenital fixation. Revision cases and those with inadequate postoperative bone or air conduction data were excluded. Inclusion criteria were met for 25 patients. Operative findings and hearing outcomes were evaluated. RESULTS: The study group was comprised of 25 stapedectomies. Closure of the air-bone gap to less than 10 dB was achieved in 48% of cases, and 80% had closure within 20 dB. A gap of more than 30 dB remained in 3 cases. There were 2 cases of sensorineural hearing loss with worsening of the bone conduction thresholds by 15 dB and 30 dB. There were no instances of perilymph gusher. In addition to an excluded case that was not reconstructed because of facial nerve position, 3 of the included patients were found to have a dehiscent facial nerve at surgery. There were no facial nerve injuries, and fixation of the malleus or incus was not found in any of the included patients. When hearing outcomes were compared with our results for otosclerosis over the same time period, rates of closure to within 10 dB and 20 dB were significantly worse in the congenital group. CONCLUSION: Closure of the air-bone gap in this population differs from our results in stapedectomy done for otosclerosis. This difference likely reflects subtle anatomic variations in the congenital group that affect the effectiveness of the prosthesis. Despite the difference in results, stapedectomy for congenital fixation remains an effective method to achieve significant hearing improvement in the majority of patients. EBM RATING: B-3b.

[Stapes fixation in children]. / Stapesfixation bei Kindern.

BACKGROUND AND OBJECTIVE: The audiometric results after stapes surgery in children with isolated stapes footplate fixation were reason enough to analyze intraoperative findings and surgical procedures. PATIENTS/METHODS: A total of 438 stapes surgeries were performed in the years 1985-2000, and a retrospective analysis was made of the anamnestic data, intraoperative findings and audiometric data from 12 children with isolated fixation of the stapes footplate. The average age of the children when operated was 10.2 years (the youngest was 7 and the eldest 13). A loss of hearing had set in 5 years earlier. RESULTS: Taking the anamnestic data and the intraoperative into account, otosclerosis was found in 5 children. One child had a narrow oval window niche, in a further six children a congenital fixation of the stapes footplate was evident. Only 58% (n=7) of the 12 children operated showed acceptable postoperative hearing results. The best hearing results were found in children with juvenile otosclerosis. In three of six children with congenital stapes fixation, a worsening of both bone and air conduction was evident. The average conductive hearing loss was 30 dB at 1, 2 and 4 kHz. CONCLUSIONS: When explorative tympanotomy is indicated, a thin-layer CT scan of the temporal bone should be made to assess the risk of a liquor pressure labyrinth developing.