The significance for postoperative hearing of preserving the labyrinth in acoustic neurinoma surgery

1992 ◽  
Vol 77 (5) ◽  
pp. 677-684 ◽  
Author(s):  
Marcos Tatagiba ◽  
Madjid Samii ◽  
Cordula Matthies ◽  
Mowaffak El Azm ◽  
Robert Schonmayr
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Semicircular Canal ◽  
Semicircular Canals ◽  
Cochlear Nerve ◽  
Acoustic Neurinoma ◽  
Content Type ◽  
Acoustic Neurinomas ◽  
A Prospective Study ◽  
Fine Print

✓ Among 186 patients with preoperative hearing, a total of 189 acoustic neurinomas were removed through a lateral suboccipital approach with anatomical preservation of the cochlear nerve. Functional hearing was preserved in 92 (49%) of these patients; despite anatomical preservation of the cochlear nerve, deafness was the result in 51 % of the series. Many factors have been considered to cause hearing loss in patients whose cochlear nerve was intact after surgery; these include nerve retraction, nerve or cochlear ischemia, overheating and vibration damage to the nerve, and opening of the labyrinth. To evaluate the significance of injury to the labyrinth in postoperative hearing loss, a prospective study was undertaken. High-resolution computerized tomography studies through the inner ear with bone algorithm were performed pre- and postoperatively. The postoperative status of the labyrinth was classified into three patterns: intact, fenestrated, and widely opened. Injury to the labyrinth occurred in 30% of the cases. The most frequently injured labyrinth structures were the crus commune of the posterior and superior semicircular canals (52%), the posterior semicircular canal (23%). the vestibule (21%), and the superior semicircular canal (4%). A statistically significant relationship was found between injury to the labyrinth and deafness, elevated thresholds, and lower discrimination values at pure-tone audiograms and speech audiometry (p < 0.0001). The degree of the injury (comparison between fenestration and wide opening of the labyrinth) was also significantly related to postoperative deafness (p < 0.0001). Disturbance of the inner-ear fluids was considered to be the cause of the hearing loss. In 12 patients labyrinth injury was not associated with deafness. This finding may support the existence of mechanisms of cochlear protection. The homeostatic function of the endolymphatic sac was considered to play an important role in recovery of damaged hearing in these 12 cases.

Ménière Recognizes That Vertigo Can Originate from the Inner Ear

2016 ◽  
Author(s):  
Robert W. Baloh
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Blood Vessels ◽  
Semicircular Canal ◽  
Semicircular Canals ◽  
Underlying Disease ◽  
The Brain ◽  
Benign Course

Prosper Ménière was the first clinician to conclude that vertigo can result from diseases of the inner ear. The symptom of vertigo originally fell under the rubric of apoplectiform cerebral congestion, a disorder thought to result from overfilling of blood vessels in the brain. Ménière noted that patients with vertigo and hearing loss associated with damage to the inner ear often have a benign course, and aggressive treatments such as bleeding can be more dangerous than the underlying disease. The first hint that the semicircular canals may be related to balance rather than hearing was provided by a Frenchman, Marie Jean Pierre Flourens. He systematically cut each semicircular canal in the pigeon and noted that the animal’s head and body tended to move in the plane of the damaged canal. The gyrations of the animals described by Flourens made Ménière think that vertigo in humans might be a similar phenomenon.

Correlation between auditory function and internal auditory canal pressure in patients with vestibular schwannomas

2002 ◽  
Vol 96 (5) ◽  
pp. 872-876 ◽  
Author(s):  
Samir B. Lapsiwala ◽  
G. Mark Pyle ◽  
Ann W. Kaemmerle ◽  
Frank J. Sasse ◽  
Behnam Badie
Keyword(s):  
Hearing Loss ◽  
Tumor Growth ◽  
Tumor Size ◽  
Internal Auditory Canal ◽  
Auditory Evoked Potential ◽  
Cochlear Nerve ◽  
Content Type ◽  
Vascular Compromise ◽  
Brainstem Response ◽  
Fine Print

Object. Hearing loss is the most common presenting symptom in patients who harbor a vestibular schwannoma (VS). Although mechanical injury to the cochlear nerve and vascular compromise of the auditory apparatus have been proposed, the exact mechanism of this hearing loss remains unclear. To test whether pressure on the cochlear nerve from tumor growth in the internal auditory canal (IAC) is responsible for this clinical finding, the authors prospectively evaluated intracanalicular pressure (ICaP) in patients with VS and correlated this with preoperative brainstem response. Methods. In 40 consecutive patients undergoing a retrosigmoid—transmeatal approach for tumor excision, ICaP was measured by inserting a pressure microsensor into the IAC before any tumor manipulation. Pressure recordings were correlated with tumor size and preoperative auditory evoked potential (AEP) recordings. The ICaP, which varied widely among patients (range 0–45 mm Hg), was significantly elevated in most patients (median 16 mm Hg). Although these pressure measurements directly correlated to the extension of tumor into the IAC (p = 0.001), they did not correlate to total tumor size (p = 0.2). In 20 patients in whom baseline AEP recordings were available, the ICaP directly correlated to wave V latency (p = 0.0001), suggesting that pressure from tumor growth in the IAC may be responsible for hearing loss in these patients. Conclusions. Tumor growth into the IAC results in elevation of ICaP and may play a role in hearing loss in patients with VS. The relevance of these findings to the surgical treatment of these tumors is discussed.

Adult aqueductal stenosis presenting with fluctuating hearing loss and vertigo

1983 ◽  
Vol 59 (4) ◽  
pp. 703-705 ◽  
Author(s):  
Orhan Barlas ◽  
Hüsameddin Gökay ◽  
M. İnan Turantan ◽  
Nermin Başerer
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Positive Contrast ◽  
Aqueductal Stenosis ◽  
The Other ◽  
Content Type ◽  
Contrast Ventriculography ◽  
Inner Ear Dysfunction ◽  
Fluctuating Hearing Loss ◽  
Fine Print

✓ Two cases of aqueductal stenosis presenting with fluctuating hearing loss, tinnitus, and vertigo are presented. Audiovestibulometric assesment of both cases disclosed the characteristic pattern of disorder of the membranous inner ear. Non-tumoral aqueductal stenosis was demonstrated by computerized tomography in one case and by positive contrast ventriculography in the other. Shunting procedures of the cerebrospinal fluid resulted in resolution of inner ear dysfunction in both patients.

Microvascular decompression for cochlear symptoms

2000 ◽  
Vol 93 (3) ◽  
pp. 421-426 ◽  
Author(s):  
Tomomi Okamura ◽  
Yasushi Kurokawa ◽  
Norio Ikeda ◽  
Seisho Abiko ◽  
Makoto Ideguchi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Microvascular Decompression ◽  
Cranial Nerve ◽  
Cochlear Nerve ◽  
Auditory Brainstem Responses ◽  
Neurovascular Compression ◽  
Minimal Risk ◽  
Eighth Cranial Nerve ◽  
Content Type ◽  
Fine Print

Object. The object of this study was to evaluate the efficacy of a new neurovascular decompression technique in relieving symptoms of cochlear nerve dysfunction.Methods. Nineteen patients with slowly progressive hearing loss, low-frequency fluctuating hearing loss, and high-pitched tinnitus due to neurovascular compression (NVC) of the eighth cranial nerve in a triangular space between the seventh and eighth cranial nerves (the VII–VIII triangle) of the cerebellopontine angle (CPA) were treated using a new technique for microvascular decompression that was developed by anatomical study in 24 cadaver specimens of the CPA. In 12 of 19 patients the anterior inferior cerebellar artery (AICA) was observed to cause compression in the VII–VIII triangle and this vessel was easily mobilized medially for placement of a silicone sponge or Teflon cushion between the compressing artery and nerve. Postoperatively, hearing loss of 20 dB or more that was present in 11 of the 19 patients with NVC improved by more than 5 dB in seven (64%), including the patient with the most severe hearing loss. Of 18 patients presenting with tinnitus preoperatively, eight (44%) had no tinnitus and an additional nine (for a total of 94%) had good improvement in tinnitus after surgery and at long-term follow up.Conclusions. The microvascular decompression technique described is highly successful in treating symptoms due to direct or indirect compression of the cochlear nerve, with minimal risk of complications. Recordings of auditory brainstem responses confirmed the clinical diagnosis of NVC of the eighth cranial nerve and correlated with clinical results after microvascular decompression of the cochlear nerve.

Delayed Hearing Loss after Surgery for Acoustic Neurinomas: Clinical and Electrophysiological Observations

Neurosurgery ◽  
1991 ◽  
Vol 28 (4) ◽  
pp. 559-565 ◽  
Author(s):  
Christian Strauss ◽  
Rudolf Fahlbusch ◽  
Johann Romstöck ◽  
Johannes Schramm ◽  
Eiju Watanabe ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Auditory Evoked Potentials ◽  
Cochlear Nerve ◽  
Acoustic Neurinoma ◽  
Pathophysiological Mechanism ◽  
Vasa Nervorum ◽  
Electrophysiological Finding ◽  
Patients At Risk ◽  
Acoustic Neurinomas ◽  
Total Deafness

Abstract In a series of 26 patients with medium-sized and large acoustic neurinomas and documented hearing before surgery, 7 patients had preservation of hearing initially after the procedure but then developed delayed hearing loss. The most prominent intraoperative electrophysiological finding in these cases was a gradual deterioration of brain stem auditory evoked potentials (BAEP);, especially of Wave V. Four additional patients with a similar gradual intraoperative loss of BAEP and severe postoperative hearing deterioration received vasoactive treatment after surgery (low-molecular weight dextran);. In all 4 patients, including 1 patient with documented total deafness after surgery, hearing was preserved. Initial preservation of cochlear nerve function after the removal of an acoustic neurinoma does not guarantee postoperative hearing. Intraoperative BAEP help to identify patients at risk for delayed postoperative hearing loss. The pathophysiological mechanism can be attributed to disturbances of the microcirculation in endoneurial vasa nervorum caused by the mechanical manipulation of the cochlear nerve.

Endolymphatic sac tumors in von Hippel—Lindau disease

2004 ◽  
Vol 100 (3) ◽  
pp. 480-487 ◽  
Author(s):  
Daniel Choo ◽  
Lawrence Shotland ◽  
Maryann Mastroianni ◽  
Gladys Glenn ◽  
Carter van Waes ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Family Members ◽  
Magnetic Resonance Images ◽  
Endolymphatic Sac ◽  
Specific Gene ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

Object. Von Hippel—Lindau (VHL) disease is a hereditary multiple-neoplasia syndrome mapping to chromosome 3p25–26. Endolymphatic sac (ELS) tumors have been identified as a neoplastic manifestation of VHL disease. The purpose of this study was to evaluate comprehensively the natural history of inner ear disease in a large population of patients with confirmed or suspected VHL disease and to correlate the clinical features with the VHL genotype. Methods. The authors collated and analyzed clinical and genotypic data obtained in patients enrolled in an Institutional Review Board—approved protocol in which families and individuals affected by VHL disease were studied. These data included results from multidisciplinary history workups and physical examinations, imaging studies, and a battery of audiological tests. One hundred seventy-five patients were enrolled in the study, 129 with confirmed VHL disease and 46 of their family members in whom test results for VHL disease were negative and who served as controls. Twenty-one patients had ELS tumors that were evident on magnetic resonance images; three of them had bilateral ELS lesions. Hearing loss, often sudden in onset and severe to profound in nature, vestibulopathy, aural fullness, and tinnitus represented the primary symptoms of ELS tumor. Distinct patterns of auditory and vestibular dysfunction occurred at different stages of the disease. Phenotypic data showed that 17 of 21 patients with ELS tumors did not have pheochromocytomas, whereas all had VHL disease affecting the kidney, all but two had VHL disease affecting the central nervous system, and all but one had disease affecting the pancreas. Genotyping revealed 10 rearrangements (partial deletions), eight single bp substitutions, and one 3-bp insertion. Although there was no difference in the incidence of hearing loss between populations, symptoms of imbalance and aural fullness were more common in patients with VHL disease but without imaging evidence of ELS tumor than they were in family members who did not have VHL disease (p < 0.01). Conclusions. Endolymphatic sac tumors are frequently associated with VHL disease. Symptoms of disequilibrium or aural fullness in patients with VHL disease may be an early indication of endolymphatic dysfunction. Patients with VHL disease provide a unique opportunity to examine the effects of specific gene mutations and a discrete neoplastic process on the human inner ear. The study of ELS tumors in this group also provides a pathological model of ELS function and supplies evidence for a role of the ELS in clinical Ménière-like disease(s).

Current results of the retrosigmoid approach to acoustic neurinoma

1992 ◽  
Vol 76 (6) ◽  
pp. 901-909 ◽  
Author(s):  
Michael J. Ebersold ◽  
Stephen G. Harner ◽  
Charles W. Beatty ◽  
C. Michel Harper ◽  
Lynn M. Quast
Keyword(s):  
Facial Nerve ◽  
Major Change ◽  
Early Postoperative Period ◽  
Activity Level ◽  
Retrosigmoid Approach ◽  
Cerebrospinal Fluid Leakage ◽  
Acoustic Neurinoma ◽  
Content Type ◽  
Acoustic Neurinomas ◽  
Fine Print

✓ Since 1984 when cranial nerve monitoring became routinely performed at the Mayo Clinic, 255 patients have undergone 256 procedures using the retrosigmoid approach for the removal of acoustic neurinomas. Of these, 221 patients had some hearing before surgery and 52 maintained hearing following surgery. The anatomical continuity of the facial nerve was preserved in 237 of these 256 procedures. It was possible to perform a primary end-to-end anastomosis in seven of the remaining 19 patients, and one patient had a cable graft inserted. Thus, 95.7% of these patients were believed to have potential for spontaneous facial nerve function. Of the 11 patients in whom this was not possible, seven underwent early spinal accessory facial anastomosis, in two hypoglossal-facial anastomosis was performed, and two had no facial nerve procedures and have paralysis of the facial nerve. There were two deaths from a pulmonary embolus in the early postoperative period, both 4 days following otherwise uneventful surgery. The most common postoperative complication was cerebrospinal fluid leakage, which has not resulted in significant permanent morbidity although early repair for this problem is now routinely recommended. Other complications were quite rare and have generally not resulted in any major change in patient lifestyle or activity level. This review reconfirms that the retrosigmoid surgical treatment of acoustic tumors continues to be an acceptable treatment option.

Cochlear nerve canal stenosis and associated semicircular canal abnormalities in paediatric sensorineural hearing loss: a single centre study

2020 ◽  
Vol 134 (7) ◽  
pp. 603-609
Author(s):  
Z Zainol Abidin ◽  
F Mohd Zaki ◽  
T Y Kew ◽  
B S Goh ◽  
A Abdullah
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Semicircular Canal ◽  
Semicircular Canals ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Bony Labyrinth ◽  
Canal Stenosis ◽  
Computed Tomography Images ◽  
Canal Diameter

AbstractObjectiveThis study aimed to evaluate the association between cochlear nerve canal dimensions and semicircular canal abnormalities and to determine the distribution of bony labyrinth anomalies in patients with cochlear nerve canal stenosis.MethodThis was a retrospective study in which high-resolution computed tomography images of paediatric patients with severe-to-profound sensorineural hearing loss were reviewed. A cochlear nerve canal diameter of 1.5 mm or less in the axial plane was classified as stenotic. Semicircular canals and other bony labyrinth morphology and abnormality were evaluated.ResultsCochlear nerve canal stenosis was detected in 65 out of 265 ears (24 per cent). Of the 65 ears, 17 ears had abnormal semicircular canals (26 per cent). Significant correlation was demonstrated between cochlear nerve canal stenosis and semicircular canal abnormalities (p < 0.01). Incomplete partition type II was the most common accompanying abnormality of cochlear nerve canal stenosis (15 out of 65, 23 per cent).ConclusionCochlear nerve canal stenosis is statistically associated with semicircular canal abnormalities. Whenever a cochlear nerve canal stenosis is present in a patient with sensorineural hearing loss, the semicircular canal should be scrutinised for presence of abnormalities.

Neurotopographic considerations in the microsurgical treatment of small acoustic neurinomas

1998 ◽  
Vol 88 (3) ◽  
pp. 506-512 ◽  
Author(s):  
Wolfgang T. Koos ◽  
J. Diaz Day ◽  
Christian Matula ◽  
David I. Levy
Keyword(s):  
Facial Nerve ◽  
Hearing Preservation ◽  
Acoustic Neurinoma ◽  
Tumor Type ◽  
Nerve Function ◽  
Speech Discrimination ◽  
Content Type ◽  
Acoustic Neurinomas ◽  
Grade Ii ◽  
Fine Print

Object. The authors studied the relationships between tumor size, location, and topographic position relative to the intact facial nerve bundles in acoustic neurinomas to determine the influence of these factors on hearing preservation postoperatively. Consistent topographic relationships were found. Methods. Four hundred fifty-two patients with acoustic neurinoma treated via a retrosigmoid approach were analyzed with respect to hearing preservation and facial nerve function. One hundred fifteen tumors were identified as small and were categorized as Grades I and II. Patients with Grade I tumors, that is, purely intracanalicular lesions, all had good hearing preoperatively, defined by a less than 50-dB pure tone average and 50% speech discrimination score. All 14 Grade I tumors were removed, resulting in preservation of the patient's hearing by these criteria. There were no particular topographic anatomical relationships associated with these tumors that affected hearing preservation. Grade II tumors, defined as those protruding into the cerebellopontine angle without contacting the brainstem, were found in 101 patients and were divided by size into two grades: IIA (< 1 cm) and IIB (1–1.8 cm). In 90 patients with Grade IIA tumors, 72 (89%) of 81 who had preserved hearing preoperatively maintained it postoperatively, and in the 11 patients with Grade IIB tumors, six of whom had good hearing preoperatively, four (67%) had preserved hearing postoperatively. Six morphological types were identified based on their neurotopographic relationships to the elements of the vestibulocochlear nerve. Conclusions. Hearing preservation postsurgery by tumor type was as follows: 1A, 92%; 1B, 88%; 1C, 100%; 2A, 83%; 2B, 92%; and 3, 57%. Combined, this represents a hearing preservation rate of 87% after surgical treatment of Grade II acoustic neurinomas. Full nerve function was maintained in 88% of patients with anatomically preserved facial nerves in both Grade I and II tumors. The remaining 12% of patients retained partial function of the facial nerve. Two patients in the series lost anatomical integrity of the nerve due to surgery.

scholarly journals Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Next Generation Sequencing ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Transverse Diameter ◽  
Congenital Hearing Loss ◽  
Canal Stenosis ◽  
Ear Anomalies ◽  
Generation Sequencing

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient’s hearing was rehabilitated with bilateral cochlear implantation.

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