Hypertrophic pachymeningitis of the internal auditory canal and cerebellopontine angle from a large recurrent attic cholesteatoma.

OBJECTIVES: To describe a rare complication of cholesteatoma. METHODS: Case report with literature review. RESULTS: We report a case of a 37-year-old male who presented for evaluation of otorrhea, headache, and progressive left sensorineural hearing loss. Clinical and radiologic evaluation demonstrated a large recurrent attic cholesteatoma with erosion into the lateral and superior semicircular canals, and diffuse enhancement of the internal auditory canal and cerebellopontine angle suggestive of hypertrophic pachymeningitis secondary to cholesteatoma. After treatment with a course of antibiotics and canal wall down mastoidectomy surgery for cholesteatoma exteriorization, he experienced improvement of his symptoms and resolution of hypertrophic pachymeningitis. CONCLUSION: Hypertrophic pachymeningitis is a rarely described complication of cholesteatoma. In the context of cholesteatoma, treatment with antibiotics and surgical removal or exteriorization of cholesteatoma are effective treatments for HP.

The Intersection Between Meritocracy and Diversity, Equity, and Inclusion.

Embracing meritocracy and diversity, equity, and inclusion is critical to the future of otolaryngology-head and neck surgery. Understanding the intersection of these two concepts is particularly important. Meritocracy, a key principle utilized in academic medicine and surgery, rewards individual achievement. However, it can inadvertently result in a widening disparity between individuals who have access to environments promoting and facilitating meritocracy and those facing systemic structural barriers. Navigating the intersection of meritocracy and diversity, equity, and inclusion is a complex endeavor. However, it is crucial to understand that these concepts can coexist. With a balanced approach, we can appreciate individual merit while fostering diversity, equity, and inclusion. It requires a commitment to systemic change, ongoing evaluation, and collaboration to create environments where everyone has an equal opportunity to succeed and contribute unique talents and perspectives. Through these efforts, our specialty of otolaryngology-head and neck surgery will be stronger.

Totally implantable hearing system: Five-year hearing results.

OBJECTIVES/HYPOTHESIS: 1) To provide long-term hearing outcome measures of a totally implantable hearing system (implant) and compare to the baseline unaided (BLU) and baseline aided (BLA) conditions, and 2) discuss relevant safety measures. STUDY DESIGN: Prospective, nonrandomized, multicenter, single-subject-as-own-control design. METHODS: Fifty-one subjects with mild to severe sensorineural hearing loss were implanted between 2008 and 2009 and enrolled in this postmarket approval study in the setting of private and hospital-based practices. Forty-nine of these subjects completed the 5-year study, which included annual follow-ups. Primary effectiveness endpoints were speech reception threshold (SRT) and word recognition scores at 50 dB (WRS50s). Secondary effectiveness endpoints were WRSs and the Abbreviated Profile of Hearing Aid Benefit (APHAB) scores. Adverse Device Effects (ADEs) and Serious Adverse Device Effects (SADEs) reported during the study period and a comparison of bone conduction scores are submitted as safety measures. RESULTS: Compared to the BLA condition, 1) SRT scores were improved at every annual follow-up; 2) WRS50s were better in 49%, and the same in 41% at the 5-year follow-up; 3) WRSs were improved by 17% at the 5-year follow-up; and 4) APHAB scores were improved in most subscales at every annual follow-up. There were three SADEs in three subjects and 15 ADEs in 11 subjects. Bone conduction scores increased by 3.7 dB at the 5-year follow-up. Average battery life was 4.9 years. CONCLUSIONS: The implant compared favorably to the subjects' hearing aid throughout the 5-year period in all of the areas measured and was found to be safe. LEVEL OF EVIDENCE: 2b. Laryngoscope, 128:210-216, 2018.

Surgical treatment of Bell's palsy: current attitudes.

OBJECTIVES/HYPOTHESIS: To learn the current management of Bell's palsy among practicing otologists and neurotologists and to better define the role of surgical decompression of the facial nerve in the treatment of Bell's palsy. STUDY DESIGN: Survey questionnaire. METHODS: We conducted a survey of members of the American Otological Society and the American Neurotology Society to learn their current practices in the treatment of Bell's palsy. RESULTS: Eighty-six neurotologists responded out of 334 surveys (26%). The majority of respondents obtain magnetic resonance imaging and electrical testing for new patients and treat with a combination of steroids and antiviral agents. More than two thirds of respondents would recommend surgery to patients who met the established electrophysiologic criteria (electroneuronography <10% normal, no spontaneous motor unit action potentials on electromyography within 10 days of onset of complete paralysis). However, only half believe that surgical decompression should be the standard of care, and only half would use a standard middle fossa approach. Lack of evidence was the most commonly cited reason for not recommending surgery. Several respondents wrote that they would leave the option of surgery to the patient. Most important, one third of neurotologists have not performed a surgical decompression for Bell's palsy in the last 10 years, and 95% perform less than one procedure per year. CONCLUSIONS: Disagreement persists among practicing otologists about the role of surgical decompression for Bell's palsy. More convincing clinical evidence will be needed before there is widespread consensus regarding the surgical treatment of this condition.

Facial Nerve Grading System 2.0.

OBJECTIVE: To present an updated version of the original Facial Nerve Grading Scale (FNGS), commonly referred to as the House-Brackmann scale. STUDY DESIGN: Controlled trial of grading systems using a series of 21 videos of individuals with varying degrees of facial paralysis. RESULTS: The intraobserver and interobserver agreement was high among the original and revised scales. Nominal improvement is seen in percentage of exact agreement of grade and reduction of instances of examiners differing by more then one grade when using FNGS 2.0. FNGS 2.0 also offers improved agreement in differentiating between grades 3 and 4. CONCLUSION: FNGS 2.0 incorporates regional scoring of facial movement, providing additional information while maintaining agreement comparable to the original scale. Ambiguities regarding use of the grading scale are addressed.

Cochlear and brainstem implantation. 2002.

Cochlear and auditory brainstem implants offer safe and effective hearing habilitation and rehabilitation for profoundly deafened adults and children. Brainstem implant technology is currently approved for use in patients with type 2 Neurofibromatosis, who have lost integrity of auditory nerves following vestibular schwannoma removal. An update on implant devices, speech processing strategies, candidacy criteria, and perceptual performance are provided in this article.

Head and neck epithelioid sarcoma in a child: diagnostic dilemma and anterolateral thigh free flap reconstruction.

The diagnostic dilemma is discussed of a child who presented with a granulomatous process of the external ear that was originally considered granuloma annulare and was later diagnosed as epithelioid sarcoma. We present the surgical treatment and the first report of reconstruction of a lateral skull base and cheek defect with an anterolateral thigh (ALT) free flap in a pediatric patient.

Cochlear and brainstem implantation.

Cochlear implantation is an established habilitative and rehabilitative option for profoundly deafened individuals over 1 year of age who derive limited benefit from conventional hearing aids. Auditory performance varies among individuals and is determined primarily by age at implantation, pre-existence of speech and language skills, and the time interval between onset of deafness and implantation. Successful implant users generally demonstrate improved auditory abilities and speech production skills beyond those achieved with hearing aids. Multichannel ABIs can provide useful auditory information to patients with NF-2 who have lost integrity of auditory nerves following removal of vestibular schwannomas. The implant allows for awareness of environmental sounds and, potentially, speech recognition. Most patients undergoing implantation demonstrate improved lip-reading skills, and exceptional performers achieve understanding of open-set speech.

Surgical management of petrous apex cholesterol granulomas.

OBJECTIVES: To review surgical approaches for drainage of petrous apex cholesterol granulomas, define the criteria for the selection of each approach, and identify factors affecting surgical outcome. STUDY DESIGN: Retrospective chart review and follow-up survey questionnaire. SETTING: Tertiary neurotologic referral center. PATIENTS: Thirty-four patients who underwent surgical treatment for symptomatic cholesterol granulomas of the petrous apex, who had a minimum of 3 months of clinical follow-up care and, in some cases, postoperative radiologic follow-up studies. INTERVENTION: Drainage with or without removal of the cyst lining was achieved through the transcanal infracochlear, transmastoid infralabyrinthine, middle fossa, translabyrinthine, and transotic approaches. Silastic catheters were used to stent drainage sites in some cases. MAIN OUTCOME MEASURES: Relief of symptoms, postoperative cranial nerve function, clinical and radiologic recurrence, and need for further surgical intervention were evaluated. Imaging studies were reviewed to determine how anatomic information influenced surgical decisions and outcome. RESULTS: Clinical improvement of symptoms was observed in 28 patients (82%), and recovery of cranial nerve function was observed in 8 of 9 affected individuals. Serviceable hearing was preserved in all but 1 patient. Five patients required revision surgery for recurrent symptoms and lesions. All 5 patients had undergone drainage procedures without the use of stents. The size of the lesion did not influence the surgical outcome. CONCLUSIONS: Determination of the appropriate surgical approach to the petrous apex is based on hearing status in the affected ear and on the anatomic relationships between the lesion and the surrounding neurovascular structures. The translabyrinthine approach is useful in nonhearing ears. In hearing individuals, anatomy permitting, the transcanal infracochlear approach with stenting is the preferred approach for drainage of petrous apex cholesterol granulomas.