Diagnosis and Management of Unexplained Conductive Hearing Loss With Intact Tympanic Membrane: A Systematic Review.

Conclusion: In patients with progressive conductive hearing loss and a normal tympanic membrane (TM), and with soft tissue density in the middle ear cavity (MEC) on temporal bone computed tomography (TBCT) scan, open-type congenital cholesteatoma (OCC) should be highly suspected and a proper surgical plan that includes mastoid exploration and second-stage operation is required. Objective: The clinical presentation of OCC is very similar to congenital ossicular anomaly (COA) presenting with a conductive hearing loss with intact TM. Therefore, it is challenging to make a correct preoperative diagnosis in patients with OCC. We evaluated the clinical characteristics of OCC compared with those of COA to find diagnostic clues useful in diagnosis of OCC. Methods: The medical records of 12 patients with surgically proven OCC and 14 patients with surgically proven COA were reviewed for demographic data, otologic history, preoperative TBCT findings, intraoperative findings, and pre- and postoperative audiologic data. Results: There was no difference between OCC and COA based on demographic data, preoperative hearing, and ossicular status on TBCT. However, the presence of progressive hearing loss, soft tissue density in the MEC on TBCT scan, and the need for mastoid surgery and second-stage operation were significantly more frequent in OCC patients.

A 44-year-old woman presented with typical vestibular symptoms of superior semicircular canal dehiscence syndrome (SSCDS). In addition, the patient experienced a rapidly progressive mixed hearing loss in the affected ear prior to surgical intervention that was unresponsive to oral steroid administration. Following middle fossa craniotomy with repair of the dehiscence, the patient's mixed hearing loss resolved to normal levels with no air-bone gap. In this report, we discuss the possible etiology of this rapidly progressive hearing loss and its implications on the differential diagnosis of patients with new onset mixed hearing losses. We also contrast the index case of progressive mixed loss with the more frequent conductive hearing loss seen in SSCDS.

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To analyze the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane, and summarize the key diagnostic points, differential diagnosis and observe the effects of surgical treatment. We reviewed data from 82 patients with unilateral conductive hearing loss with intact tympanic membranes who accepted the exploratory tympanotomy from April 2011 to September 2013. There were 41 males and 41 females, aged from 7 to 66( averaged 26.5±13.7)years, with a history of one month to 50 years. The history, clinical symptoms, audiological evaluation, high resolution temporal bone CT, the results of surgical exploration and hearing reconstruction were analyzed. The exploratory tympanotomy revealed 43 cases of congenital middle ear malformations (52.4%), 22 cases of otosclerosis (26.8%), eight cases of congenital cholesteatoma (9.8%), six cases of trauma induced conductive hearing loss (7.3%), three cases of congenital ossicular malformations with congenital cholesteatoma (3.7%). Progressive hearing loss was common in patients with otosclerosis and congenital cholesteatoma, and patients with congenital middle ear malformations described their hearing loss since childhood. High resolution temporal bone CT of congenital middle ear malformation, trauma induced conductive hearing loss, congenital cholesteatoma diagnosis rate was 40.0%, 50.0%, and 83.3% respectively. The preoperative air-conductive threshold of patients with absence of the oval window were increased to (66.9±1.1)dBHL, the preoperative bone-conductive threshold achieved (28.3±10.4)dBHL at 2 000 Hz. While patients with stapes fixation and that with ossicular chain discontinuity were (27.2±9.7)dBHL and (17.8±8.8)dBHL(P=0.000)respectively. Through the tympanic exploration with endaural incision under the microscope, different hearing reconstruction were applied according to different lesions. After the operation, the hearing level of 52 patients with return visit were improved, the mean air-conductive threshold were decreased from (60.0±11.4)dBHL to (32.2±12.1)dBHL(P=0.000); and the mean ABG were decreased from (43.2±12.0)dB to (16.3±9.4)dB(P=0.000). Congenital middle ear malformations, otosclerosis, congenital cholesteatoma are the most common causes in unilateral conductive hearing loss with an intact tympanic membrane. The diagnosis rate can be improved by analyzing the clinical features. Through exploratory tympanotomy and hearing reconstruction, we can clarify the diagnosis and achieve a satisfying hearing recover.

Is Preoperative Computed Tomography Necessary or Useful for Primary Stapes Surgery?

Patient: Male, 52-year-oldFinal Diagnosis: Superior semicircular canal dehiscence • tegmen tympani dehiscenceSymptoms: Dizziness • hearing loss • vertigo • vomitingClinical Procedure: —Specialty: RadiologyObjective:Rare diseaseBackground:Superior semicircular canal dehiscence is an inner-ear pathology which presents with vertigo, disequilibrium, and hearing loss. Although the exact etiology of superior semicircular canal dehiscence is unknown, it is thought that an increase in middle-ear pressure disrupts a thin overlying temporal bone. Superior semicircular canal dehiscence is frequently seen in association with dehiscence of the tegmen tympani, which overlies the middle ear. Here, we present a case report of a 52-year-old Puerto Rican man with vertigo, dizziness, vomiting, and mild hearing loss associated with superior semicircular canal and tegmen tympani dehiscence after performing improper scuba diving techniques.Case Report:A 52-year-old Puerto Rican man presented to the emergency department with vertigo, dizziness, vomiting, and mild hearing loss in the right ear. The symptoms began shortly after scuba diving with inadequate decompression techniques on ascent. He was treated with recompression therapy with mild but incomplete improvement in symptoms. Bilateral temporal magnetic resonance imaging was suggestive of segmental dehiscence of the right superior semicircular canal and tegmen tympani. High-resolution computed tomography of the temporal bone confirmed right superior semicircular canal and tegmen tympani dehiscence with an intact left inner ear.Conclusions:The increased inner-ear pressure that occurs during scuba diving can lead to dehiscence of the superior semi-circular canal and tegmen tympani, causing vertigo and hearing loss. Performance of improper diving techniques can further increase the risk of dehiscence. Therefore, appropriate radiologic evaluation of the inner ear should be performed in such patients.

Objective:To explore the clinical efficacy of transcanal endoscopic ear surgery in the diagnosis and treatment of conductive hearing loss with intact tympanic membrane. Method:The clinical data of 16 patients with conductive hearing loss with intact tympanic membrane were retrospectively analyzed. They were diagnosed and treated by transcanal endoscopic ear surgery. Result:All patients were diagnosed by exploratory tympanotomy, including 6 cases of congenital middle ear anomalies, 5 cases of congenital cholesteatoma, 2 cases of congenital middle ear anomalies with congenital cholesteatoma, 2 cases of otosclerosis, and 1 case of traumatic ossicular chain disruption. During the tympanic exploration by transcanal endoscopic ear surgery, different methods of hearing reconstruction were applied according to the intraoperative lesions. Among 14 cases（14 ears）, 7 patients underwent reconstruction with partial ossicular replacement prosthesis （PORP）, 5 patients had total ossicular replacement prosthesis （TORP）, and 2 patients had piston. The remaining 2 patients did not undergot ossicular reconstruction. After the operation, the mean air-conductive threshold of 14 patients decreased from （61.7±6.5） dB HL to （29.8±10.7） dB HL （P<0.01） and the mean ABG decreased from（36.8±3.2） dB HL to （10.7±6.9） dB HL （P<0.01）. 1 case of congenital middle ear anomalies with congenital cholesteatoma underwent the lesion resection without ossicular reconstruction. Due to lack of suitable Piston, 1 case of congenital middle ear anomalies with fixed stapes did not perform hearing reconstruction. No serious complications occured after operations. Conclusion:Transcanal endoscopic ear surgery was suitable for the diagnosis and treatment of conductive hearing loss with intact tympanic membrane. It was minimally invasive with low complications, and the patients had a good hearing recovery after ossicular reconstruction.

Choristoma is a mass of tissue histologically normal for an organ or part of the body other than the site at which it is located. A rare case of ectopic salivary gland choristoma in the middle ear is described in a 14-year-old girl whose only symptom was a 55 dB conductive hearing loss. The left middle ear mass appeared whitish and was located behind the intact tympanic membrane. We first suspected it to be congenital middle ear cholesteatoma. Exploratory tympanotomy, however, revealed a salivary gland choristoma that adhered tightly to the facial nerve. Differential diagnosis and treatment of this rare condition are discussed.

To aggregate symptoms reported by patients with superior canal dehiscence syndrome (SCDS) and to develop an evidence-based symptom set by performing a systematic review of the literature. Database search and critical assessment of research studies. Medline and PubMed databases were searched for articles that reported the preoperative symptoms of adult and pediatric patients with unilateral and bilateral SCDS. Articles were excluded if they reported on associated diseases or did not report symptoms. Of the 397 articles retrieved, 66 were retained for quantitative analysis. Among 431 patients with SCDS, 91 symptom terms were reported. After combining synonymous terms, 22 symptoms were derived by consensus. Of the raw total number of reported symptoms, 92.5% can be attributed to five common symptoms: spontaneous dizziness (51%), autophony (42.5%), pressure-induced vertigo (37.4%), hearing loss (39.9%), and sound-induced vertigo (42.7%). This systematic review of symptoms reported by patients with SCDS identified a 22-item common symptom set. These items can be used to create an evidence-based patient-reported outcome measure to evaluate health-related quality of life in SCDS. Laryngoscope, 1932-1938, 2018.

Congenital cytomegalovirus infection inducing non-congenital sensorineural hearing loss during childhood; a systematic review

We report a case of a patient with superior semicircular canal dehiscence syndrome, a recently described condition in which vestibular imbalance and/or hearing loss results from the discontinuity of the bone overlying the superior semicircular canals. Case report. A 46-years-old women presented with autophony in the left ear and imbalance when shouting (Tullio phenomenon). Temporal bone computer tomography revealed a defect of the left superior semicircular canal caused by an enlarged superior petrosal sinus receiving drainage from a large cerebellar developmental venous anomaly. Conclusion. We review superior semicircular canal dehiscence syndrome and its management, and we discuss common aetiologies. We conclude that superior semicircular canal dehiscence syndrome may present with a solely developmental aetiology, despite presenting late in life.

Superior semicircular canal dehiscence syndrome has recently been reported as a cause of pressure- or sound-induced oscillopsia (Tullio phenomenon). We report the presentation and successful treatment of 3 patients with superior semicircular dehiscence syndrome by a joint neurosurgical/neuro-otology team. Patient 1 is a 37-year-old man who presented with complaints of disequilibrium, fullness in the left ear, hearing loss, and oscillopsia when pressure was applied to the left external auditory canal. Patient 2 is a 46-year-old man who presented with complaints of disequilibrium, fullness in the left ear, and blurred vision associated with heavy lifting or straining. On examination, pneumatic otoscopy produced a sense of motion. Patient 3 is a 29-year-old woman who presented with chronic disequilibrium that resulted in frequent falls. She had a positive fistula test on the left, and vertical nystagmus was elicited when pressure was applied to the left ear. In each patient, high-resolution computed tomographic scanning through the temporal bone revealed dehiscence of the superior semicircular canal on the symptomatic side. In all 3 cases, a subtemporal, extradural approach was performed with repair of the middle fossa floor using calcium phosphate BoneSource (Howmedica Leibinger, Inc., Dallas, TX). All patients recovered well, with resolution of their symptoms. Superior semicircular canal dehiscence syndrome is a cause of disequilibrium associated with sound or pressure stimuli. The workup includes a detailed history, electronystagmography including Valsalva maneuvers, and a high-resolution computed tomographic scan though the temporal bone. An extradural repair of the middle fossa floor with BoneSource can successfully treat this condition.

Profile of childhood hearing loss in the Western Cape, South Africa

To describe the presentations and treatment results for patients with concurrent otosclerosis and superior semicircular canal dehiscence (SCD). Retrospective case series and literature review. Tertiary academic medical center. Patients with concurrent diagnoses of otosclerosis (radiographically and/or surgically confirmed) and SCD (radiographically confirmed) in the same ear. Review of medical records. Clinical presentations, outcomes following stapedotomy. Eight patients with 10 affected ears were identified. All patients presented with slowly progressive conductive hearing loss, normal otoscopy, absent acoustic reflexes, and without other symptoms of SCD syndrome. Seven patients were treated with stapedotomy and 1 with hearing aids. Of those treated with stapedotomy, a persistent conductive hearing loss was the most common hearing result. One patient had near-complete closure of their air bone gap. None had a profound sensorineural hearing loss. Four patients had unmasking of SCD symptoms. The clinical and audiometric presentations of patients with concurrent otosclerosis and SCD are often indistinguishable from those of patients with only otosclerosis. Computed tomography of the temporal bone is the only way to identify concurrent SCD. Stapedotomy in these patients typically results in a persistent conductive hearing loss, though 14 to 33% of patients experience near-complete closure of their air bone gap. SCD symptoms are unmasked in 57 to 63% of patients who undergo stapedotomy. Further work is needed to delineate the utility of routine preoperative computed tomography scan in otosclerosis patients, and to identify prognostic factors for patients with concurrent otosclerosis and SCD who wish to undergo stapedotomy.

Superior semicircular canal dehiscence syndrome (SCDS) is a clinical entity resulting in a myriad of audiological and vestibular symptoms. Pressure and/or sound-induced vertigo/nystagmus, autophony, conductive hearing loss, and conductive hyperacusis are commonly seen in patients with SCDS. The physiologic mechanism of this syndrome is thought to be due to the dehiscence creating a low-impedance outlet for fluid waves in the labyrinth, commonly referred to as a third window. This shunts flow from the cochlea to the labyrinth, which both activates the vestibular system and decreases pressure driving the traveling fluid wave in the cochlea. Diagnosis of SCDS can be difficult and requires both radiographic evidence of a dehiscence and clinical evidence supporting SCDS as the etiology for a patient’s symptoms. Testing for SCDS includes high-resolution CT imaging, audiogram, cervical and ocular VEMP testing, head impulse testing, and visualization of sound- or pressure-induced eye movements in the plane of the affected superior canal. Traditional surgical repair of SCDS is via the middle fossa approach. This approach allows for the dehiscence to be seen directly and for it to be both plugged and resurfaced. This approach is preferred in the vast majority of patients, but in certain situations a transmastoid approach can offer benefits over the middle fossa approach. Surgical outcomes for repair of SCDS are quite favorable, with patients having improvement of autophony, imbalance, and vertigo. Patients with predominately audiological symptoms have the highest likelihood of postoperative improvement. The majority of patients report an improvement in their quality of life postoperatively. Complications are rare but do occur in both the middle fossa and transmastoid approaches to SCDS repair. The most common complication is hearing loss, which is typically mild but can be profound in a low percentage of patients. The importance of proper patient selection and preoperative counseling on the risks of surgery cannot be overstated to ensure good surgical outcomes in SCDS repair.