Emberger syndrome: A rare association with hearing loss.

Emberger Syndrome (ES) is a rare genetic disorder characterized by lymphedema and myelodysplasia. It is also associated with hearing loss. The genetic mutations associated with ES are not part of the comprehensive 80 gene next generation sequencing (NGS) panel. As a result, the otolaryngologist should maintain an index of suspicion for ES in any child with SNHL who presents repeatedly with recurrent infections, lymphedema and/or cutaneous warts. This paper describes the clinical evolution and management of two children who were followed up for hearing loss and eventually were diagnosed with ES.

Current and future techniques for human papilloma virus (HPV) testing in oropharyngeal squamous cell carcinoma.

Despite a reduction in smoking and alcohol consumption, the incidence of oropharyngeal squamous cell carcinoma (OPSCC) is rising. This is attributed to human papilloma virus (HPV) infection and screening for HPV is now recommended in all cases of OPSCC. Despite a variety of clinically available tests and new non-invasive test strategies there is no consensus on which technique is best. This review reports on current techniques for HPV detection in OPSCC and the clinical applicability of emerging techniques. Literature searches of Medline, Embase and clinicaltrials.gov using the search terms 'head and neck neoplasms', 'squamous cell carcinoma' and 'HPV testing' were performed. 45 studies were identified and included. p16 immunohistochemistry (IHC), HPV DNA in situ hybridization (ISH) and HPV polymerase chain reaction (PCR) are the commonest tests to determine HPV status. p16 IHC and HPV DNA PCR are highly sensitive whilst HPV DNA ISH is more specific, these techniques conventionally utilize surgical biopsies. New tests using PCR to screen fine needle aspirates, saliva, brush cytology and serum for HPV are promising but have variable sensitivity and specificity. These non-invasive samples avoid the morbidity of surgical biopsies and need for tissue blocks; their clinical role in screening and surveillance remains largely untested. Further work is needed to validate these tests and define their role.

Middle ear gentamicin-soaked pledgets in the treatment of Ménière's disease.

OBJECTIVE: Assess the relative efficacy and results of the round window hyaluronic acid pledget depot method for intratympanic gentamicin delivery in Ménière's disease. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral hospital. PATIENTS: Prospective symptomatic and audiologic data were collected on 28 patients undergoing intratympanic gentamicin therapy for Ménière's disease refractory to medical treatment between 2003 and 2009. INTERVENTION: All patients had the round window membrane exposed via a tympanomeatal flap, and any adhesions were removed. Hyaluronic acid pledgets soaked in 40 mg/ml of gentamicin were then packed into the round window niche and posterior mesotympanum, and the tympanomeatal flap was replaced. MAIN OUTCOME MEASURES: Patients audiologic and vertigo symptom outcome scores were recorded at follow-up according to the AAO-HNS 1995 guidelines for reporting results in Ménière's disease. RESULTS: Complete or substantial improvement in vertigo (class A or B) was achieved in 88.5% (23/26) of patients. Hearing loss, defined as greater than 10 dB (PTA average at 0.5, 1, 2, and 4 kHz) was noted in 12 (50%) of 24 patients including 4 patients who had developed "dead ears" (16%). The average hearing loss for all patients excluding the 4 patients with dead ears was 10.7 dB. CONCLUSION: Round window hyaluronic acid pledget technique used as a sustained delivery vehicle for intratympanic gentamicin treatment for Ménière's disease produces similar rates of vertigo control compared with other techniques but a greater risk of hearing loss. We would recommend the intratympanicinjection titration technique as first line for most patients.