Edwin M. Monsell

The Mechanism of Hearing Loss in Paget's Disease of Bone†

Objectives/Hypothesis The mechanism of hearing loss (HL) in Pagets disease of bone was investigated. The present study was a systematic, prospective, controlled set of clinical investigations to test the hypothesis that there is a general underlying mechanism of HL in Pagets disease of bone and to gain additional insights into the auditory and otologic dynamics of this disease. Specific questions were 1) whether the mechanism is cochlear or retrocochlear and 2) whether the bone mineral density of the cochlear capsule is related to hearing levels.

The Cooperative Outcomes Group for ENT: A multicenter prospective cohort study on the outcomes of tympanostomy tubes for children with otitis media

OBJECTIVE: Outcomes for patients with otitis media were assessed in this prospective, multicenter study. STUDY DESIGN AND SETTING: Thirty-one otolaryngologists enrolled 272 pediatric patients with otitis media; caregivers completed surveys at 3-month intervals, and clinical and treatment data was also collected. The Otitis Media 6 (OM-6) was the primary outcome measure. RESULTS: One hundred seventy-seven patients (mean age 2.0 years) completed 3-month follow-up. One hundred thirty-seven patients underwent tympanostomy tube placement. Large improvements in disease-specific quality of life (QOL) were seen up to 9 months of follow-up. Baseline OM-6 score was the best predictor of clinical success in regression modeling. CONCLUSIONS: Patients referred to an otolaryngologist for treatment of otitis media see large improvements in disease-specific QOL regardless of treatment rendered. SIGNIFICANCE: The study demonstrates the feasibility of multicenter outcomes studies and confirms appropriate triage of patients with otitis media into surgical versus medical interventions. EBM rating: C.

Glioblastoma multiforme after microsurgery for acoustic neuroma without radiotherapy: limitations of the Cahan criteria.

In 1948 Cahan and others reported 11 cases of sarcoma that arose in radiated bone, mostly in children treated for tuberculosis. They studied only cases that met certain inclusion criteria: 1) there must have been histologic or radiographic evidence of the nonmalignant nature of the initial condition; 2) the sarcoma that arose must have arisen in the area included in the radiotherapeutic beam; 3) there must have been a relatively long, asymptomatic period of latency between radiation and development of the sarcoma; and 4) the sarcoma must be proved histologically. Since Cahan’s report, these criteria have often been cited as requirements for considering a case to represent radiationinduced malignancy. Since the 1980s there has been considerable interest in stereotactic radiosurgery and other forms of focused radiation therapy for nonmalignant conditions. One of the concerns raised by radiation treatments for acoustic neuroma, which has somewhat limited their use, is the potential for radiation to induce other intracranial tumors, including malignancies. Because such cases are rare, it has been difficult to establish a reliable estimate of their occurrence to guide selection of treatment and patient counseling. We report a case that we feel should be taken into consideration when estimating the risk of radiation-induced malignancy. Institutional Review Board approval was obtained. A 58-year-old woman presented to the emergency department complaining of increasing ataxia for the past two weeks. She also noted numbness and tingling in the right face and body, diplopia, blurred vision, nausea, and vomiting for the same period. On the morning of admission she experienced a severe pressure headache at the right mastoid process, which spread to include the left side within a few hours. The past medical history was significant for microsurgical excision of a right acoustic neuroma two years previously. This was conducted as a two-stage procedure at another center. A few months postoperatively she received an osseointegrated implant for single-sided deafness. The patient’s family history was negative for cancer, schwan-

The Cooperative Outcomes Group for ENT: A multicenter prospective cohort study on the effectiveness of medical and surgical treatment for patients with chronic rhinosinusitis:

OBJECTIVE: To assess outcomes for patients with chronic rhinosinusitis. STUDY DESIGN AND SETTING: Prospective, multicenter study; 31 otolaryngologists enrolled 276 adult patients with chronic rhinosinusitis; patients completed surveys at 3-month intervals, and physicians provided clinical and treatment data. The primary outcome measure was the Chronic Sinusitis Survey-Duration (CSS-D). RESULTS: One hundred seventeen adult patients completed 12-month follow-up. Most patients reported sinus symptoms lasting longer than 3 years (74%). Patients showed significant improvement (group P < 0.0001) in the CSS-D score at each follow-up interval. Baseline CSS-D (P < 0.0001), surgical intervention (P < 0.003), and Lund-McKay score (P < 0.04) were predictive of clinical success in regression analysis. CONCLUSIONS: Patients referred to an otolaryngologist have a severe sinus illness. Treatment by an otolaryngologist was associated with significant improvement in sinus-related symptoms. SIGNIFICANCE: The study demonstrated the feasibility of multicenter outcome studies in chronic rhinosinusitis and generated testable hypotheses for future investigation. EBM rating: C.

Extracts from The Cochrane Library: vestibular rehabilitation for unilateral peripheral vestibular dysfunction (review).

The “Cochrane Corner” is a quarterly section in the Journal that highlights systematic reviews relevant to otolaryngology—head and neck surgery, with invited commentary to highlight implications for clinical decision making. This installment features a Cochrane Review entitled “Vestibular rehabilitation for unilateral peripheral vestibular dysfunction,” which concludes that there is moderate to strong evidence from high-quality randomized trials supporting the safety and efficacy of this intervention.

Emerging Concepts of Hearing Loss in Paget's Disease of Bone

Hearing loss has been recognized as a clinical feature of Paget’s disease of bone since Sir James Paget’s first case report (1). Involvement of the skull is commonly associated with involvement of the temporal bones and with hearing loss in Paget’s disease (2). An older concept that hearing loss in Paget’s disease is due to multiple mechanisms is giving way to a newer concept that there is a general mechanism causing hearing loss in most cases (3). Clinical and audiometric studies of hearing loss in Paget’s disease have described high-frequency sensorineural hearing loss and a low-frequency air–bone gap in most cases (4). (An air–bone gap indicates that auditory thresholds when measured by bone conduction are better than those predicted by air conduction thresholds.) An air–bone gap is usually interpreted as indicating a conductive type of deafness. Hearing levels have been shown to progress more rapidly in untreated Paget’s disease than in uncomplicated presbycusis (5). Histologic studies in the 1960s and 1970s showed a variety of derangements, including compression and stretching of the auditory nerve, microfractures of the otic capsule, loss of auditory hair cells and ganglion cells, invasion of labyrinthine spaces, and envelopment of ossicles by pagetic bone (4,6,7). More recently, histopathologic studies have also shown atrophy of the spiral ligament and endolymphatic hydrops (8). The presence of toxic cytokines has been shown in otosclerosis and suggested in Paget’s disease. Most of these data consist of histopathologic reports of advanced cases. On the other hand, the prominent histopathologist of the temporal bone, Harold F. Schuknecht, had long held the view that such dramatic findings were not the rule. He analyzed the largest group of histopathologic cases of Paget’s disease of the temporal bone to date in 1990 (9). Using a technique of graphic analysis that compared hearing levels in the range of the speech frequencies versus the loss of auditory hair cells and ganglion cells that carry the neural place code for each of those tones, he could not demonstrate any systematic relationships. Aside from the presence of pagetic bone, the specimens were typical for the age of the subjects. There were no findings that would explain the air-bone gap, such as fixation or disruption of the auditory ossicles.