Malleus bar: an unusual ossicular abnormality in the setting of congenital aural atresia.

OBJECTIVES: Malleus bar is a rare cause of conductive hearing loss and has yet to be described in the setting of congenital aural atresia. This study aimed to define the anomaly and review the surgical management and outcomes of patients found to have a malleus bar in the setting of congenital aural atresia. STUDY DESIGN: Retrospective case review of patients with malleus bar and congenital aural atresia. SETTING: Tertiary otologic referral center. PATIENTS: Patients found to have a malleus bar at the time of surgery for congenital aural atresia. METHODS: Charts of patients who underwent surgery for congenital aural atresia repair with a malleus bar were reviewed for demographic data, preoperative Jahrsdoerfer score, surgical findings, and audiometric data. RESULTS: Seven subjects (8 ears) were identified to have a malleus bar and congenital aural atresia. In all 8 ears, the malleus bar proved to be a second point of attachment of the ossicular chain in addition to the atretic plate. The chorda tympani nerve was found in the bony bar in 4 patients. Closure of the air-bone gap within 35 dB hearing level (HL) was achieved in 83% of patients. All patients showed improved speech reception thresholds and stable bone-conduction thresholds. All but 1 patient had stable or improved word recognition scores. CONCLUSION: Malleus bar in the setting of congenital aural atresia represents a rare combination of causes of congenital conductive hearing loss. Patients with these 2 abnormalities obtain good short-term results with surgical repair of congenital aural atresia.

To POP or not: ossiculoplasty in congenital aural atresia surgery.

OBJECTIVES/HYPOTHESIS: To examine indications for ossiculoplasty (OP) in aural atresia surgery and to compare audiometric results and surgical revision rates between patients undergoing OP and those undergoing intact native chain reconstruction (INCR). STUDY DESIGN: Retrospective chart review. METHODS: Charts of patients undergoing surgery for congenital aural atresia were reviewed for demographic data, preoperative Jahrsdoerfer score, ossicular chain status, and audiometric data. Patients undergoing OP were compared with an equal number of age and Jahrsdoerfer grade-matched patients who had an INCR. The preoperative and postoperative average air-bone gap (ABG), speech reception thresholds (SRT), and rates of revision surgery were compared between the two groups. RESULTS: Nineteen patients (20 ears) underwent OP during aural atresia repair and were compared with 20 matched patients who had INCR. Mean age, Jahrsdoerfer score, preoperative ABG, and SRT were similar for both groups. Mean postoperative audiometric follow-up was 33.1 months for the OP group and 20.4 months for the INC group (P = .24). Mean postoperative ABG was 33.8 dB HL for OP and 23.8 dB HL for INCR (P < .05). Mean improvement in ABG was 16.8 dB HL for OP and 29.9 dB HL for INCR (P < .001). Mean improvement in SRT was 24.6 dB HL for OP and 34.8 dB HL for INCR (P < .05). Nine ears (45%) in the OP group and four ears (20%) in the INCR group underwent revision surgery (P = .09). CONCLUSIONS: Patients reconstructed with their own intact native chain during aural atresia surgery have better audiometric outcomes than those undergoing OP and are less likely to undergo revision surgery.

Congenital absence of the oval window: diagnosis, surgery, and audiometric outcomes.

OBJECTIVE: To review the surgical management and outcomes of patients with congenital absence of the oval window (CAOW) undergoing the oval window drill-out (OWD) procedure. STUDY DESIGN AND SETTING: A retrospective chart review of patients with CAOW seen in a tertiary care otologic practice between 1991 and 2006. PATIENTS/INTERVENTION: Patients with CAOW undergoing OWD procedure. MAIN OUTCOME MEASURES: Findings at surgery and preoperative and postoperative audiometric outcomes (air and bone conduction thresholds; speech reception threshold). RESULTS: Seventeen ears underwent OWD for CAOW. Four cases were aborted. For the 13 subject ears, average 1-month postoperative 4-tone air conduction and speech reception thresholds were significantly lower than preoperative thresholds. Long-term follow-up (average, 20 months) air conduction and speech reception thresholds were also significantly lower but showed a slight worsening of thresholds compared with the 1-month postoperative results. Speech discrimination scores and bone conduction thresholds remained stable. CONCLUSION: OWD may be a viable operation for patients with CAOW, although longer follow-up is needed. Given the mixed hearing results in the current patient series, we have tempered our enthusiasm for this operation. Technically challenging, this procedure nevertheless can improve patients' hearing with minimal morbidity. Revision surgery did not result in improved outcome.

Congenital cholesteatoma in a case of congenital aural atresia.

OBJECTIVE: A congenital cholesteatoma arising in the setting of congenital aural atresia is described. This represents the first case reported in the literature. STUDY DESIGN: Case report. SETTING: Tertiary otologic referral center, University of Virginia Health System, Charlottesville, Virginia. PATIENT: Eight-year-old patient with unilateral congenital aural atresia. INTERVENTIONS: Preoperative computer tomographic scanning and surgical atresia repair with removal of cholesteatoma. MAIN OUTCOME MEASURES: Recurrence of cholesteatoma; epithelialized ear canal; hearing improvement. RESULTS: A congenital cholesteatoma located medial to the ossicular mass in a child with congenital aural atresia was removed, with no evidence of recurrent disease at a revision operation 1 year later. Hearing was improved to a speech reception threshold of 25 dB. CONCLUSION: Congenital cholesteatoma can arise medial to the ossicular mass in congenital aural atresia. This finding lends support to the epidermoid rest theory for the cause of congenital cholesteatoma.

Diagnosis and management of salivary fistula after surgery for congenital aural atresia.

OBJECTIVE: Salivary fistula is an uncommon and unreported yet meaningful complication associated with the repair of congenital aural atresia. The capsule of the parotid gland may be violated during two steps of the operation: the initial dissection around the glenoid fossa or while aligning the auricle with the bony canal at the end of the procedure. We present the first described series of patients with salivary fistula after repair of congenital aural atresia. STUDY DESIGN: Retrospective case review from 1985 to 2004. SETTING: Tertiary referral center. PATIENTS: We included all patients who were diagnosed with a salivary fistula after congenital aural atresia repair. MAIN OUTCOME MEASURE: The diagnosis of a salivary fistula or salivary tissue in the external auditory canal after atresia repair was based on one of the following criteria: 1) identification of a fistula tract or salivary tissue in the external auditory canal, 2) otorrhea positive for amylase, or 3) intermittent otorrhea associated with eating. RESULTS: Of 1,500 patients operated on for aural atresia, we identified 6 with salivary fistula after atresia repair. Salivary fistulas were diagnosed from 15 days to 10 years postoperatively, and the duration ranged from 6 months to 14 years. Treatment included observation, medical management, and surgical intervention. CONCLUSION: Salivary fistulas in the external auditory canal may present with granulation tissue, persistent crusting, or persistent otorrhea; it is therefore necessary to consider salivary fistula when managing these findings in postoperative congenital aural atresia patients. Salivary fistula secondary to repair of congenital aural atresia may be managed conservatively or surgically.

Congenital auricular atresia: update on options for intervention and timing of repair.

Although the surgical correction of congenital atresia is difficult, it has the potential to achieve a level of patient and physician satisfaction unparalleled in other types of ear surgery.