Venous Outflow and Otosclerosis: An Unusual Case of Pulsatile Tinnitus.

We report resolution of right-sided pulsatile tinnitus in a 44-year-old male who underwent stapedectomy for fenestral otosclerosis. Initial workup revealed a mixed hearing loss and absent stapedial reflexes consistent with ossicular fixation. CT angiography demonstrated near complete stenosis of the left-sided transverse and sigmoid sinuses and dominant contralateral venous outflow. We hypothesized that the dominant right cerebral venous outflow tract created turbulent flow that was conducted to cochlea. Successful stapedectomy was performed, and the pulsatile tinnitus resolved. This case report demonstrates evidence that the sound of turbulent blood flow can be conducted through bone and an instance where the perception of vascular tinnitus was eliminated with stapedectomy.

Long-Term Hearing Results of Endoskeletal Ossicular Reconstruction in Chronic Ears Using Titanium Prostheses Having a Helical Coil: Part 1-Kraus K-Helix Crown, Incus to Stapes.

OBJECTIVES: 1) To assess long-term hearing results after endoskeletal ossicular chain reconstruction (eOCR) using the titanium Kraus K-Helix Crown prosthesis, implanted incus to stapes, with glass-ionomer cement (GIC) in chronic ears and 2) to determine safety of the prosthesis and cement. STUDY DESIGN: Prospective, nonrandomized, sequential, single center, single surgeon. SETTING: Private practice, ambulatory surgical center. PATIENTS: N = 15 males (42%) and 21 females (58%). Mean age was 40.4 years (range, 6-81 years); 38 ears (22 right ears [58%] and 16 left ears [42%]). INTERVENTIONS: eOCR in chronic ears. Comprehensive preoperative and postoperative hearing measurements were performed for up to 9 years. MAIN OUTCOME MEASURES: Postoperative hearing results at 1 year showed statistically significant improvement as compared with preoperative hearing. Long-term hearing results remained stable and showed no statistically significant change over 9 years. RESULTS: Estimated mean pure-tone air conduction average improved by 14.5 dB (95% confidence interval = 10.3-18.7). Estimated mean speech reception thresholds improved by 15.5 dB (10.8-20.2). Word recognition scores improved by -2.2% (-5.3 to 1.0). The estimated mean postoperative air-bone gap was 10.5 dB (7.2-13.8). The estimated mean calculated air-bone gap was 11.3 dB (8.0-14.5). The estimated mean change in high-tone bone conduction (HTBC) average was +3.5 dB (0.9-6.0). Two prostheses extruded (5%). No patients experienced any unanticipated serious adverse effects or events. CONCLUSION: eOCR using the K-Helix Crown prosthesis, incus to stapes, and GIC can significantly improve hearing at 1 year and maintain stable hearing over 9 years. Both prosthesis and cement are safe.

Scaly, circular papules in a healthy toddler.

A 2-year-old boy presents with asymptomatic keratotic papules on his hands and feet. A biopsy is performed and he is confirmed to have punctate porokeratotic keratoderma. A discussion of punctate porokeratotic keratoderma is provided along with treatment options.

Endoskeletal ossicular reconstruction using the Kraus K-Helix Crown and Piston middle ear prostheses.

The Kraus K-Helix Crown and Piston prostheses are novel, newly designed, MRI compatible, titanium ossicular replacement prostheses that have received U.S. Food and Drug Administration clearance in March of 2008 for ossiculoplasty. Reconstruction is indicated to restore sound conduction of the ossicular chain during tympanoplasty in chronic and non-chronic ears. The prostheses have been specifically developed for reconstruction of an eroded or absent long process of the incus and an eroded or absent stapes superstructure. They may be used with or without glass-ionomer cement. The prostheses may be implanted incus-to-stapes or malleus-to-stapes (K-Helix Crown) or may be implanted incus-to-footplatelneo-membrane or malleus-to-footplate/neo-membrane (K-Helix Piston). The K-Helix Piston is useful during revision stapedectomy when an eroded long process of the incus is encountered. By coating the K-Helix prostheses with glass-ionomer cement, it is possible to perform "endoskeletal ossicular reconstruction" which greatly enhances long-term prosthesis stability and avoids contact with the tympanic membrane. The prostheses may be custom-fit to reconstruct each individual patient's anatomy. Preliminary hearing results in a small cohort of patients at one year indicate very satisfactory hearing results with closure of the air-bone gap in the majority of patients. The ENT OR nurse plays a role in the use of specialized, adjunctive OR equipment that is used with the technology, is required for the preparation of glass-ionomer cement, and provides patients with important educational information concerning reconstructive otologic procedures.

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.

An unusual case of acute foot drop caused by a pseudoaneurysm.

Lower extremity neurologic symptoms are a common presenting problem. Here we report the case of a 73-year-old man who developed acute right foot pain and foot drop. History, physical examination, and electrodiagnostic studies were consistent with a lumbosacral plexopathy. Imaging studies revealed an internal iliac artery pseudoaneurysm, a rare cause of acute foot drop.

Envoy Esteem Totally Implantable Hearing System: phase 2 trial, 1-year hearing results.

OBJECTIVES: (1) To assess outcomes of the Envoy Esteem Totally Implantable Hearing System as measured by hearing results compared with preimplant baseline unaided (BLU) and best-fit aided conditions (BLA) and (2) to determine safety of the device. STUDY DESIGN: Prospective, nonrandomized, multicenter, subject-as-own-control, US Food and Drug Administration (FDA) trial. SETTING: Private practice and hospital-based. SUBJECTS AND METHODS: Between January 2008 and August 2009, an FDA trial was performed at 3 sites. Fifty-seven subjects with bilateral, mild to severe sensorineural hearing loss, with discrimination greater than 40%, were implanted. Implanted components were (1) a sound processor and (2) 2 piezoelectric transducers (a sensor and a driver). A sound processor was implanted in the temporal bone. Transducers were coupled to the ossicles. Devices were activated 2 months postimplant. Hearing results were compared with ipsilateral BLU and BLA. RESULTS: Speech reception thresholds (SRTs) improved from BLA of 41.2 dB to 29.4 dB with the Esteem (P ≤ .001). Word recognition score (WRS) at 50 dB hearing level (HL) improved from BLA of 46.3% to 68.9% with the Esteem. Pure tone averages improved by 27 ± 1 dB (confidence interval, 30-25). There were no changes in bone conduction. QuickSIN results showed no change. There were 6 serious adverse device effects: 2 wound infections (1 resolved medically, 1 required explantation), 1 delayed facial paralysis that resolved with medication, and 3 revisions due to limited benefit. CONCLUSION: Phase 2 results at 12 months post implant demonstrated that (1) hearing results with the device are statistically superior to baseline best-fit hearing aids for SRT and WRS and (2) the device is safe.

Profound high-frequency sensorineural hearing loss treatment with a totally implantable hearing system.

OBJECTIVE: This study seeks to assess the efficacy of the Envoy Esteem totally implantable hearing device in treating profound high-frequency sensorineural hearing loss. PATIENTS: Five patients with profound high-frequency hearing loss participating in a prospective, multi-center, nonrandomized Food and Drug Administration clinical trial. INTERVENTION: Implantation with Esteem totally implantable hearing device and comparison to baseline unaided and aided conditions. MAIN OUTCOME MEASURE: Speech reception threshold and word recognition scores (WRS) at 50 dB HL presentation level. RESULTS: Preoperative speech reception threshold improved from an unaided 65 dB and aided 48 dB average to 26 dB with the Esteem at 12 months. WRS at 50 dB scores improved from an unaided 10% and aided 23% average to 78% postoperatively. CONCLUSION: The Esteem totally implantable middle ear hearing device provides appreciable functional gain and improvement in WRS to rehabilitate hearing in patients with a profound high-frequency sensorineural hearing loss.

Medical malpractice and rhinology.

BACKGROUND: Physicians facing malpractice litigation are in uncharted territory. The language, concepts, rules, and strategies of the legal system are foreign to science-based physicians. Understanding the statistics of rhinology malpractice litigation may aid the physician to cope with the assault of a claim. METHODS: Information from the 2006 Physician Insurers Association of America (PIAA) and the 2006 PIAA Risk Management Report (RMR)-Otorhinolaryngology were searched for claims data referable to the nose, nasal chamber, and paranasal sinuses. The PIAA data sharing report (DSR) is the largest single resource of malpractice claims data containing both settlement and trial judgment information. RESULTS: The nose, nasal cavity, and paranasal sinuses represent nearly two-thirds of the total indemnity paid for improper performance from otolaryngology head and neck surgery (Oto-HNS) medical malpractice claims between 1985 and 2005 based on claims information. Improper performance accounts for 50.3% of total monies paid ($107.6 million of $213.6 million) to resolve Oto-HNS claims in this period. Of nearly 2400 operative claims closed in the period of 1985-2005, 34.1% involved procedures on the nose and sinuses. In the past 6 years, $103.5 million indemnity has been added to the otolaryngology total. CONCLUSION: The area of endoscopic sinus surgery has substantially contributed to Oto-HNS claims in the PIAA DSR. Despite malpractice being a cost of medical practice, every claim is perceived as an assault on the surgeon's competence. Ensuring informed consent and complete documentation may assist the provider in defending their care.

Perivascular myoma of myopericytoma and myofibromatosis-type arising in a chronic scar.

We describe a case of a cutaneous perivascular myoma with features overlapping between the myofibromatosis and the myopericytoma type. The patient is a 58-year-old woman with a painless plaque-like and multinodular lesion in the pretibial dermis and subcutaneous tissue. She had repeated trauma to this site, first in her early youth that left an area of hyperpigmentation, and then again at age 40. The biopsy showed a biphasic pattern with a myofibromatosis-type component composed of spindle cell myoid nodules and more cellular round cell areas. The myopericytoma-like areas appeared to be infiltrating along vessels. These areas contained aggregates of immature-appearing cells arranged concentrically around vascular lumina in a manner reminiscent of pericytes. Immunohistochemical stains showed focal positivity for smooth muscle actin. Immunohistochemical and ultrastructural studies have showed these pericyte-like cells to be of a myoid origin. The reason for the neoplastic proliferation of perivascular myoid cells is presently unknown. The association of trauma and neoplastic transformation of the skin is rare. We report the first case of a cutaneous perivascular myoma arising in a chronic scar.

Bilateral anterior granulomatous uveitis associated with cutaneous Rosai-Dorfman disease.

BACKGROUND: Rosai-Dorfman disease (RDD) is a benign, rare, histiocytic disorder of unknown etiology. We describe the case of a 61-year-old woman who presented to the ophthalmology clinic with bilateral granulomatous anterior uveitis. METHODS: The uveitis was controlled on topical prednisolone acetate 1%. One month after presentation, she developed generalized macules and papules, and dermatologic and systemic evaluations were performed. RESULTS: On the basis of skin biopsy, purely cutaneous RDD was established. CONCLUSION: Anterior granulomatous uveitis may be the presenting sign of cutaneous RDD. Consultation is necessary for accurate diagnosis.

Cutaneous tumors as the initial presentation of non-T, non-B, nonmyeloid CD4+ CD56+ hematolymphoid malignancy in an adolescent boy.

We describe a 14-year-old Hispanic boy who presented with a 2-month history of enlarging plum-colored cutaneous tumors on his face, trunk, and proximal extremities. Histopathologic examination showed nodular infiltrates of malignant mononuclear cells extending from the superficial dermis to the deep subcutis. Immunohistochemical staining of the biopsy specimen and flow cytometry studies on a bone marrow aspirate revealed a CD4+, CD56+ hematolymphoid tumor that was negative for all other myeloid and lymphoid markers. Based on this information, the patient was diagnosed with the recently described, rare non-T, non-B, nonmyeloid CD4+ CD56+ hematolymphoid malignancy. To our knowledge, this is the youngest patient reported in the literature.

Adult onset of eruptive widespread Spitz's nevi.

Cases of multiple Spitz's nevi are rare, usually occurring in the agminate form or even less commonly as widespread eruptive Spitz's nevi. Previously reported cases of widespread eruptive Spitz's nevi arose in persons age 23 or younger. We describe a Hispanic male patient with eruptive Spitz's nevi that presented at the age of 35 years.

Paraneoplastic movement disorder in a patient with non-Hodgkin's lymphoma and CRMP-5 autoantibody.

The paraneoplastic autoantibody, collapsin response-mediator protein (CRMP)-5 immunoglobulin G (IgG), is specific for neuronal cytoplasmic CRMP-5, and is usually associated with small-cell lung carcinoma or thymoma. We report on details of a movement disorder that followed anti-B-cell therapy in a patient with lymphoma, and was accompanied by CRMP-5 IgG.