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Selective Stapedial tendon and Tensor Tympani tenotomy for the treatment of Middle Ear Myoclonus in a pediatric patient

Objective tinnitus is a rare phenomenon whereby a patient perceives sound in the absence of external auditory stimuli, that is also observed by the examiner. Unlike subjective tinnitus which is thought to be somatosensory and usually difficult to cure, objective tinnitus is more likely to have an identifiable cause amenable to treatment. The differential for objective tinnitus includes aberrant vascular anatomy affecting the temporal bone, patulous eustachian tube function, and abnormal myoclonic activity of the palatal or middle ear muscles.1  

We present a 16-year-old female who presented for evaluation of objective tinnitus. On physical examination, an intermittent rhythmic clicking was identified. Visualization of both the tympanic membrane and palate during active audible tinnitus was observed and found to be normal. A hearing test was performed demonstrating normal hearing and speech thresholds as well as normal tympanogram. Acoustic reflex testing demonstrated absent decay in both ears and  spontaneous discharge for the right ear in response to both high and very low stimulus indicating abnormal stapedial and tensor tympani function. MRA demonstrated normal vascular anatomy and MRI was obtained demonstrating normal anatomy without lesions of the brainstem, cochleovestibular nerves, or ear or mastoid pathology. The patients was subsequently diagnosed with isolated middle ear myocolonus (MEM). Treatment options including medical versus surgical therapy were discussed as has previously been described. The patient ultimately elected for surgical tenotomy of the stapedial and tensor tympani tendons. Using endoscopic technique, a middle ear exploration was performed. Canal injection was performed with standard tympanomeatal flap elevation was assisted with epinephrine pledgets. The Annular ligament was identified and the middle ear was entered. Additional dissections was performed superiorly, and the chorda tympani nerve was identified and preserved. The stapedial tendon was visualized emanating from the pyramidal eminence to the posterior crus of the stapes. Balluci scissors were used to sharply incise the tendon and the remaining ends were reflected using a Rosen needle to prevent re-anastamosis. Additional dissection along the malleus was performed to gain access to the tensor tympani tendon. A 30 degree angled endoscope was utilized to visualize the tensor tympani tendon extending forward from the cochleariform process to the neck of the malleus. The angled 6400 Beaver blade was used to sharply incise the tendon, requiring multiple passess due to the thickness of the tendon. The sharply incised ends of both tendons were clearly visualized. The tympanomeatal flap was re draped and secured with gel foam packing. The patient was seen in follow up three weeks post operatively with a well healed ear drum, resolution of her objective tinnitus, normal hearing, and absent stapedial reflexes. The patient and mother were happy. Endoscopic stapedial and tensor tympani tenotomy is a feasible technique for isolated MEM in the pediatric population.

Title: Selective Stapedial tendon and Tensor Tympani tenotomy for the treatment of Middle Ear Myoclonus in a pediatric patient   Sean Evans, MD1,2 1 Emory University School of Medicine, Department of Otolaryngology, Atlanta, GA, USA 2 Children’s Healthcare of Atlanta, Division of Otolaryngology, Atlanta, GA, USA Keywords: (FIVE) objective tinnitus; myoclonus; middle ear myoclonus; stapedial tendon tenotomy; tensor tympani tenotomy   Educational/Technical Point(s): Middle ear tenotomy is a feasible technique for the treatment of middle ear myoclonus in the pediatric population Case Presentation: Objective tinnitus is a rare phenomenon whereby a patient perceives sound in the absence of external auditory stimuli, that is also observed by the examiner. Unlike subjective tinnitus which is thought to be somatosensory and usually difficult to cure, objective tinnitus is more likely to have an identifiable cause amenable to treatment. The differential for objective tinnitus includes aberrant vascular anatomy affecting the temporal bone, patulous eustachian tube function, and abnormal myoclonic activity of the palatal or middle ear muscles.1   We present a 16-year-old female who presented for evaluation of objective tinnitus. On physical examination, an intermittent rhythmic clicking was identified. Visualization of both the tympanic membrane and palate during active audible tinnitus was observed and found to be normal. A hearing test was performed demonstrating normal hearing and speech thresholds as well as normal tympanogram. Acoustic reflex testing demonstrated absent decay in both ears and  spontaneous discharge for the right ear in response to both high and very low stimulus indicating abnormal stapedial and tensor tympani function. MRA demonstrated normal vascular anatomy and MRI was obtained demonstrating normal anatomy without lesions of the brainstem, cochleovestibular nerves, or ear or mastoid pathology. The patients was subsequently diagnosed with isolated middle ear myocolonus (MEM). Treatment options including medical versus surgical therapy were discussed as has previously been described. The patient ultimately elected for surgical tenotomy of the stapedial and tensor tympani tendons. Using endoscopic technique, a middle ear exploration was performed. Canal injection was performed with standard tympanomeatal flap elevation was assisted with epinephrine pledgets. The Annular ligament was identified and the middle ear was entered. Additional dissections was performed superiorly, and the chorda tympani nerve was identified and preserved. The stapedial tendon was visualized emanating from the pyramidal eminence to the posterior crus of the stapes. Balluci scissors were used to sharply incise the tendon and the remaining ends were reflected using a Rosen needle to prevent re-anastamosis. Additional dissection along the malleus was performed to gain access to the tensor tympani tendon. A 30 degree angled endoscope was utilized to visualize the tensor tympani tendon extending forward from the cochleariform process to the neck of the malleus. The angled 6400 Beaver blade was used to sharply incise the tendon, requiring multiple passess due to the thickness of the tendon. The sharply incised ends of both tendons were clearly visualized. The tympanomeatal flap was re draped and secured with gel foam packing. The patient was seen in follow up three weeks post operatively with a well healed ear drum, resolution of her objective tinnitus, normal hearing, and absent stapedial reflexes. The patient and mother were happy. Endoscopic stapedial and tensor tympani tenotomy is a feasible technique for isolated MEM in the pediatric population.
Objective tinnitus is a rare phenomenon whereby a patient perceives sound in the absence of external auditory stimuli, that is also observed by the examiner. Unlike subjective tinnitus which is thought to be somatosensory and usually difficult to cure, objective tinnitus is more likely to have an identifiable cause amenable to treatment. The differential for objective tinnitus includes aberrant vascular anatomy affecting the temporal bone, patulous eustachian tube function, and abnormal myoclonic activity of the palatal or middle ear muscles.
We present a 16-year-old female who presented for evaluation of objective tinnitus. On physical examination, an intermittent rhythmic clicking was identified. Visualization of both the tympanic membrane and palate during active audible tinnitus was observed and found to be normal. A hearing test was performed demonstrating normal hearing and speech thresholds as well as normal tympanogram. Acoustic reflex testing demonstrated absent decay in both ears and  spontaneous discharge for the right ear in response to both high and very low stimulus indicating abnormal stapedial and tensor tympani function. MRA demonstrated normal vascular anatomy and MRI was obtained demonstrating normal anatomy without lesions of the brainstem, cochleovestibular nerves, or ear or mastoid pathology. The patients was subsequently diagnosed with isolated middle ear myocolonus (MEM). Treatment options including medical versus surgical therapy were discussed as has previously been described. The patient ultimately elected for surgical tenotomy of the stapedial and tensor tympani tendons. Using endoscopic technique, a middle ear exploration was performed. Canal injection was performed with standard tympanomeatal flap elevation was assisted with epinephrine pledgets. The Annular ligament was identified and the middle ear was entered. Additional dissections was performed superiorly, and the chorda tympani nerve was identified and preserved. The stapedial tendon was visualized emanating from the pyramidal eminence to the posterior crus of the stapes. Balluci scissors were used to sharply incise the tendon and the remaining ends were reflected using a Rosen needle to prevent re-anastamosis. Additional dissection along the malleus was performed to gain access to the tensor tympani tendon. A 30 degree angled endoscope was utilized to visualize the tensor tympani tendon extending forward from the cochleariform process to the neck of the malleus. The angled 6400 Beaver blade was used to sharply incise the tendon, requiring multiple passess due to the thickness of the tendon. The sharply incised ends of both tendons were clearly visualized. The tympanomeatal flap was re draped and secured with gel foam packing.
The patient was seen in follow up three weeks post operatively with a well healed ear drum, resolution of her objective tinnitus, normal hearing, and expectedly absent unilateral stapedial reflexes.
Endoscopic stapedial and tensor tympani tenotomy is a feasible technique for isolated MEM in the pediatric population.
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Lanska DJ. Subjective tinnitus. MedLink Neurology. 2006 Mar 13. Hidaka H, Honkura Y, Ota J, Gorai S, Kawase T, Kobayashi T. Middle ear myoclonus cured by selective tenotomy of the tensor tympani: strategies for targeted intervention for middle ear muscles. Otology & Neurotology. 2013 Dec 1;34(9):1552-8.

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