Bilateral Vestibular Schwannomas in a Patient with Ring Chromosome 22: Case Report and Review of the Literature.

INTRODUCTION: Ring chromosome 22 (r[22]) can lead to the development of intracranial tumors such as meningiomas, neurofibromas, and schwannomas similar to neurofibromatosis 2 (NF2). CASE PRESENTATION: An 18-year-old female with r(22) and a history of global development delay and cognitive impairment presented with sudden hearing loss. MRI revealed bilateral vestibular schwannomas. Given documented audiologic decline in the patient's hearing, the larger tumor was treated with CyberKnife fractionated stereotactic radiosurgery, and the smaller tumor is being monitored. CONCLUSION: This case provides further evidence that patients with r(22) can develop clinical features of NF2, including the development of bilateral vestibular schwannomas, and should be monitored for hearing disturbances starting in puberty as a warning sign for these tumors.

Complication from hearing aid mold material: a case report and review of legal matters.

Ear mold impression middle ear foreign bodies are a rare complication of hearing aid fitting. Only a small number of cases have been reported; however, the actual incidence is unknown and likely much higher than expected. We present the case of a 77-year-old man with a history of preexisting tympanic membrane perforations who presented with an ear mold impression middle ear foreign body that required surgical removal. We explore the state and federal laws that guide the interaction between patients, hearing aid sellers and otolaryngologists. We highlight steps that can be taken to reduce the incidence of this complication.

Balloon dilation of the eustachian tube for dilatory dysfunction: A randomized controlled trial.

OBJECTIVES/HYPOTHESIS: To assess balloon dilation of the Eustachian tube with Eustachian tube balloon catheter in conjunction with medical management as treatment for Eustachian tube dilatory dysfunction. STUDY DESIGN: In this prospective, multicenter, randomized, controlled trial, we assigned, in a 2:1 ratio, patients age 22 years and older with Eustachian tube dilatory dysfunction refractory to medical therapy to undergo balloon dilation of the Eustachian tube with balloon catheter in conjunction with medical management or medical management alone. METHODS: The primary endpoint was normalization of tympanogram at 6 weeks. Additional endpoints were normalization of Eustachian Tube Dysfunction Questionaire-7 symptom scores, positive Valsalva maneuver, mucosal inflammation, and safety. RESULTS: Primary efficacy results demonstrated superiority of balloon dilation of the Eustachian tube with balloon catheter + medical management compared to medical management alone. Tympanogram normalization at 6-week follow-up was observed in 51.8% (72/139) of investigational patients versus 13.9% (10/72) of controls (P < .0001). Tympanogram normalization in the treatment group was 62.2% after 24 weeks. Normalization of Eustachian Tube Dysfunction Questionaire-7 Symptom scores at 6-week follow-up was observed in 56.2% (77/137) of investigational patients versus 8.5% (6/71) controls (P < .001). The investigational group also demonstrated substantial improvement in both mucosal inflammation and Valsalva maneuver at 6-week follow-up compared to controls. No device- or procedure-related serious adverse events were reported for those who underwent balloon dilation of the Eustachian tube. CONCLUSIONS: This study demonstrated superiority of balloon dilation of the Eustachian tube with balloon catheter + medical management compared to medical management alone to treat Eustachian tube dilatory dysfunction in adults. LEVEL OF EVIDENCE: 1b. Laryngoscope, 128:1200-1206, 2018.

Interleukin-8 production in response to tumor necrosis factor-alpha by cholesteatoma keratinocytes in cell culture.

OBJECTIVES/HYPOTHESIS: Keratinocytes harvested from acquired cholesteatoma and grown in cell culture will demonstrate increased interleukin-8 (IL-8) production in response to tumor necrosis factor (TNF)-alpha as compared with a control keratinocyte cell line. Immunohistochemical studies have identified IL-8 and TNF-alpha, mediators of bony destruction, in tissue samples of cholesteatoma. TNF-alpha stimulates IL-8 production in healthy epidermal keratinocyte cell lines. It is not known whether TNF-alpha stimulates IL-8 production in cultured cholesteatoma keratinocytes. STUDY DESIGN: Prospective controlled tissue culture experiment. METHODS: Tissue from an acquired cholesteatoma was dissociated and grown in keratinocyte serum-free media for 8 weeks. Cholesteatoma keratinocytes and a control cell line of skin epidermal keratinocytes were treated with TNF-alpha. Conditioned media were harvested; production of IL-8 was measured by enzyme-linked immunosorbent assay, and cell counts were performed. RESULTS: At a zero concentration of TNF-alpha, mean production of IL-8 by cholesteatoma keratinocytes was 39,809 pg/mL/24hr/1 × 10(6) cells versus 1,907 pg/mL/24hr/1 × 10(6) cells from skin epidermal keratinocytes, a statistically significant difference (P < .05). The cholesteatoma keratinocytes showed a 2.1-fold increase in response to 2 pg/mL of TNF-alpha and a 2.44-fold increase in response to 20 pg/mL of TNF-alpha. The skin epidermal keratinocyte cell line demonstrated a 1.07- and 1.13-fold increase to respective concentrations of TNF-alpha. CONCLUSIONS: Cholesteatoma keratinocytes appear to retain cell signaling characteristics in vitro that distinguish them from skin epidermal keratinocytes. This finding may indicate that cholesteatoma keratinocytes undergo a change in behavior in vivo that is preserved after the cells are removed from the inflammatory environment of the middle ear.

Late failure rate of hearing preservation after middle fossa approach for resection of vestibular schwannoma.

OBJECTIVE: To explore the long-term hearing results after a middle fossa approach for resection of vestibular schwannoma. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: All patients undergoing a middle fossa approach for resection of vestibular schwannoma at a single institution with intent to preserve hearing between December 1989 and December 2009 were included in the study population. INTERVENTIONS: Standard middle fossa approach for resection of vestibular schwannoma, magnetic resonance imaging (MRI), and audiogram. MAIN OUTCOME MEASURES: Recurrence of tumor as evaluated by MRI and hearing results as measured by serial audiograms. RESULTS: Seventy-eight patients were identified who met study criteria. Fifty-one (65%) of the 78 patients had usable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] class A or B) postoperatively. Forty-four patients with successful hearing preservation had follow-up beyond their initial postoperative visit. Five (11%) of the 44 patients showed late degradation to nonserviceable hearing (AAO-HNS class C or D) over a mean follow-up of 4.0 years. Kaplan-Meier estimate of preservation of class A or B hearing at 10 years was 72%. Of the five patients with late degradation in hearing, 2 were found to have recurrences of their original tumor on MRI. There were 5 confirmed recurrences in the total study population. CONCLUSION: Late degradation of hearing was an infrequent occurrence after initially successful hearing preservation. When hearing degradation did occur, there seemed to be a correlation with tumor recurrence.

Implantable hearing devices.

PURPOSE OF REVIEW: Implantable hearing devices have been developed to address the shortcomings of traditional hearing aids. Currently, there are two US Food and Drug Administration clinical trials evaluating fully implantable hearing devices. RECENT FINDINGS: Implantable hearing devices attempt to improve upon stigma, cosmesis, occlusion effect, and feedback, as well as other factors that prevent patients from using traditional hearing aids. However, there are many challenges inherent in designing an implantable hearing device. Different devices have attempted to overcome these challenges in different ways. We will discuss these differences in design, their individual pros and cons as well as directions of future research. SUMMARY: Although implantable hearing devices are not currently available they do hold promise for patients not adequately served by conventional hearing aids.