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A case of papillary endolymphatic sac tumor
Otolaryngology Head and Neck Surgery P202
Scientific Posters
sule is defined pathologically as a thick enveloping layer of connective tissue that is both microscopically and macroscopically evident, it must be concluded that ANs are nonencapsulated, at least in the conventional sense of the term. The surface peel observed intraoperatively is most likely surgically created during tumor debulking by cleaving the looser central component from the more compressed portion that lies immediately beneath the free margin of the lesion. 145
Intracranial Complications of Chronic Ofitis Media PRISCILA BOGAR, MD, ALESSANDRA RAMOS VENOSA, MD, FABIANA GON(~ALEZ, MD, and RICARDO FERREIRABENTO, MD, PHD, S00 Paulo, Brazil
Intracranial complications resulting from chronic otitis media are unusual; however, they are often serious. In this paper we describe five patients with intracranial complications of chronic otitis media who were hospitalized during 1994. All patients had sigmoid sinus thrombophlebitis, three had extradural abscess, two had bacterial meningitis, and one had a brain abscess. Usually two or more intracranial complications are found. In our series all patients but one had suppurative otitis media. All of the complications were caused by granulation tissue; only one had a congenital cholesteatoma eroding the semicircular canal, but during the surgery we found that a granulation tissue was causing the erosion to the posterior fossa. One patient was human immunodeficiency virus-positive. We emphasize that some complications are quite subtle and that acute exacerbation of a chronic infection is the usual precursor of complications. When a complication is suspected, a high-resolution computed tomography (CT) scan is mandatory. The etiologic factors, CT findings, bacteriology, surgical approach, and results are discussed.
August 1995
147 Brainstem Evoked Auditory Potential in Neurology and Neurosurgery LUIZC. A, SOUSA, SADY S. DA COSTA, MANOEL J. DE ANDRADE, and MARCELO R. T. PIZA, RibeirOo Prato and Porto Alegre, Brazil
Brainstem evoked auditory potential (ABR) is the choice method to analyze the integrity of the auditory pathways, from the cochlear nerve to the inferior colliculum. In the past decade, it has also been used by neurologists and neurosurgeons for early diagnosis, follow-up, and monitoring of degenerative, inflammatory, vascular, and tumoral diseases that involve the brain stem. Besides allowing us electrophysiologic access to the brain stem, it is a noninvasive, practical, and reliable method that can be used at the patient's bedside. Working with neurologists and neurosurgeons who have started to use ABR as a method to analyze the integrity of the patient's brainstem auditory pathways, we could identify several patients whose pathologies were at a very early stage, due to delays of fractions of milliseconds in their ABR wave pattern. We want to present some cases in which ABR was conclusive in order to continue the work-up of these patients, where after radiologic imaging (computed tomography and/ or magnetic resonance imaging) we could recognize retrocochlear lesions, such as microneurinoma of the acoustic nerve, multiple sclerosis, arachnoid cyst, adrenoleukodistrophy, vestibular neuronitis, hydrocephalus, brainstem infarctation, and internal carotid anomaly.
148 A Case of Papillary Endolymphatic Sac Tumor WON-SANG LEE, MD, and HO-KI LEE,MD, Seoul, Korea
146
Otolaryngologic Manifestations of Creutzfeldt-Jakob Disease--A Case Report: Creutzfeldt-Jakob Disease Presenting as Hearing Loss and Dizziness DOUGLAS C. BIGELOW, MD, DAVID E. SCHMIDT, MD, DAVID M. YEN, and GLEN W. KNOX, MD, Philadelphia, Pa.
Creutzfeldt-Jakob Disease (CJD) is a progressive fatal neurodegnerative disorder. One of the transmissible spongiform encephalopathies, this disease is caused by prions, an abnormal isoform of a normal host protein, and is unique in that it can occur as an inherited, sporadic, or acquired disease. Clinical findings provide the only clue to early stages of CJD. Because cerebellar abnormalities will develop in many of these patients, some may present to the otolaryngologist with dizziness. Hearing loss, however, is extremely rare. We describe a patient with CJD who presented with progressive hearing loss and subsequent dizziness. A review and discussion of the otolaryngologic manifestations of CJD is presented.
Papillary tumor of the endolymphatic sac is a very rare, silently aggressive neoplasm with extensive temporal bone destruction. A 34-year-old woman was seen with complaints of tinnitus and progressive hearing impairment in the right ear for 1 year and right facial weakness for 6 months. The eardrums were intact, but a puretone audiogram showed normal hearing in the left ear and a 60 dB sensorineural heating loss in the right ear. The speech discrimination score was 14%, and a caloric test revealed 35% canal paresis on the right ear. Right facial weakness was grade III according to the HouseBrackmann classification, and a 53% degenerative response was noted on electromyogram. Computerized tomography showed a retrolabyrinthine tumor with an irregular margin and prominent intratumoral bony spicules, and magnetic resonance imaging showed a heterogeneous mass with hyper-, hypo-, and isointense foci with T1 and T2 weighting. The tumor invaded the labyrinth and the mastoid portion of the fallopian canal. Angiography revealed that the tumor was hypervascular and was fed by ascending pharyngeal, occipi-
Otolaryngology Head and Neck Surgery Volume 113 Number 2
tal, and anterior inferior cerebellar arteries. Right-side mastoid exploration was done after embolization of the feeding vessels. Profuse bleeding was noted during dissection. The tumor contained fragile bone pieces and invaded the mastoid portion of the facial nerve and sigmoid sinus. The tumor was completely removed using a translabyrinthine and petrosal approach. The pathologic diagnosis was a papillary adenoma of endolymphatic sac. The patient is free of disease 5 months after treatment. 149
Eustachian Tube Obstruction (ETO) in Cats and Chinchillas: Preliminary Results MARCELO R. DETOLEDO PIZA, MD, PATRICIA A. SCHACHERN, MICHAEL M. PAPARELLA, MD, and SHINICHI SANO, MD, RibeirOo Preto, Brazil, and Minneapolis, Minn.
Of 18 cats and 27 chinchillas studied, bilateral eustachian tube obstruction (ETO) was performed in 15 cats and 23 chinchillas. Three cats and four chinchillas without obstruction or histologic evidence of otitis media were used as controis, and one obstructed animal from each species was excluded because of other pathologies. The animals were killed 2 days and 1, 2, and 4 weeks after surgery, respectively. After processing of the temporal bones, adjacent sections 20 Ix thick were stained for hematoxylin and eosin, alcian blue (pH 2.5), periodic acid-Schiff and alcian blue (pH 2.5)periodic acid-Schiff and studied under light microscopy. Quantitative and qualitative studies were done at specific areas of the protympanum, round window niche, and promontory in cats, and at the protympanum, round window niche, and apex of the cochlea in chinchillas. The gross histologic findings, number of goblet and ciliated cells, subepithelial space thickness, and staining characteristics of the goblet cells were studied. Our conclusions were as follows: (1) In the chinchilla the development of middle ear effusions was poor when compared with cats; (2) cats developed mucoid effusion, while the chinchilla developed serous or seropurulent effusions; (3) the inflammatory process was more generalized and intense in cats, affecting the middle ear mucosa as a whole, whereas in chinchillas it is mostly restricted to the protympanum area; (4) no granulation tissue was observed in the chinchilla, but new bone formation was common; (5) the total number of goblet cells in the chinchilla is much smaller than in cats, in normal and eustachian tube obstructed animals; (6) there was a tendency of the goblet cells to secrete more acid glycoproteins than neutral at the late stages of ETO in both species. The findings of this study demonstrate that the changes of the middle ear in cats with otitis media are more similar to those of humans than in the chinchilla. Furthermore, the cat is the species of choice for the study of mucoid otitis media.
Scientific Posters P203
150
The Auditory and Vestibular Manifestations of Charcot Marie-Tooth Disease DANIEL R. FEAR, MD, C. BRUCE MACDONALD, MD, and L. CLARKE COX, PHD, Boston, Mass.
Objective: To present the auditory-vestibular manifestations of Charcot Marie-Tooth Disease (CMT). Case Report: A 53-year-old woman complained of progressive imbalance over 1 year. Imbalance was described as the sense that she was about to fall to one side, was exacerbated by rapid head acceleration, and had no relation to postural changes. Symptoms were continuous, and she fell multiple times. She used a cane for 1 year, but for the last month required a walker. Bilateral tinnitus and heating loss developed over the last year. CMT had been diagnosed about 20 years earlier, with progressive distal limb weakness a n d wasting and peripheral sensory neuropathy. The neurotologic examination was normal except for the following: There was mild tremor in the left hand. Unsupported stance was unsteady, and she fell with eye closure. Vibrotactile sensation was reduced at the hands and ankles. Audiometry demonstrated a mild, somewhat asymmetric sensorineural hearing loss (SNHL). Auditory brainstem response showed poor waveform morphology bilaterally; however, the latencies for waves III and IV were within the normal range. Electronystagmography with rotary chair data showed hypofunction and reduced vestibulo-ocular gain, Ophthalmologic evaluation was refused. Discussion: CMT is an autosomal-dominant disorder manifested by progressive peripheral neuropathy, resulting in distal muscle atrophy and a peripheral sensory neuropathy. SNHL has been described in CMT. The Rosenberg-Chutorian Syndrome involves CMT with SNHL and optic atrophy. Other cranial neuropathies have also been described in CMT. Results: We describe a patient with CMT patient who had hearing loss and imbalance. The combination of vestibulopathy, sensory (proprioceptive) neuropathy, and muscle atrophy produced a devastating balance disorder. The literature on otologic manifestations of CMT is reviewed. 151
Facial Paralysis Following Otic Drop Usage SILVIO MARRA, MD, JOHN P. LEONETTI,MD, and SUSAN FOWLES, RN, Maywood, Ill.
Six days after undergoing a right superficial parotidectomy, a 59-year-old woman complained of ipsilateral otalgia. Although her parotid wound and facial movement appeared normal, the patient was noted to have otomycosis of the right external auditory meatus. After appropriate debridement, the patient was given acetic-boric Drops (Domeboro solution) and was instructed to return in 7 days. The patient returned in 48 hours with excruciating otalgia after placement of the drops and obvious incomplete facial paresis. This progressed to a total facial paralysis over the ensuing 2 days.
Scientific Posters
sule is defined pathologically as a thick enveloping layer of connective tissue that is both microscopically and macroscopically evident, it must be concluded that ANs are nonencapsulated, at least in the conventional sense of the term. The surface peel observed intraoperatively is most likely surgically created during tumor debulking by cleaving the looser central component from the more compressed portion that lies immediately beneath the free margin of the lesion. 145
Intracranial Complications of Chronic Ofitis Media PRISCILA BOGAR, MD, ALESSANDRA RAMOS VENOSA, MD, FABIANA GON(~ALEZ, MD, and RICARDO FERREIRABENTO, MD, PHD, S00 Paulo, Brazil
Intracranial complications resulting from chronic otitis media are unusual; however, they are often serious. In this paper we describe five patients with intracranial complications of chronic otitis media who were hospitalized during 1994. All patients had sigmoid sinus thrombophlebitis, three had extradural abscess, two had bacterial meningitis, and one had a brain abscess. Usually two or more intracranial complications are found. In our series all patients but one had suppurative otitis media. All of the complications were caused by granulation tissue; only one had a congenital cholesteatoma eroding the semicircular canal, but during the surgery we found that a granulation tissue was causing the erosion to the posterior fossa. One patient was human immunodeficiency virus-positive. We emphasize that some complications are quite subtle and that acute exacerbation of a chronic infection is the usual precursor of complications. When a complication is suspected, a high-resolution computed tomography (CT) scan is mandatory. The etiologic factors, CT findings, bacteriology, surgical approach, and results are discussed.
August 1995
147 Brainstem Evoked Auditory Potential in Neurology and Neurosurgery LUIZC. A, SOUSA, SADY S. DA COSTA, MANOEL J. DE ANDRADE, and MARCELO R. T. PIZA, RibeirOo Prato and Porto Alegre, Brazil
Brainstem evoked auditory potential (ABR) is the choice method to analyze the integrity of the auditory pathways, from the cochlear nerve to the inferior colliculum. In the past decade, it has also been used by neurologists and neurosurgeons for early diagnosis, follow-up, and monitoring of degenerative, inflammatory, vascular, and tumoral diseases that involve the brain stem. Besides allowing us electrophysiologic access to the brain stem, it is a noninvasive, practical, and reliable method that can be used at the patient's bedside. Working with neurologists and neurosurgeons who have started to use ABR as a method to analyze the integrity of the patient's brainstem auditory pathways, we could identify several patients whose pathologies were at a very early stage, due to delays of fractions of milliseconds in their ABR wave pattern. We want to present some cases in which ABR was conclusive in order to continue the work-up of these patients, where after radiologic imaging (computed tomography and/ or magnetic resonance imaging) we could recognize retrocochlear lesions, such as microneurinoma of the acoustic nerve, multiple sclerosis, arachnoid cyst, adrenoleukodistrophy, vestibular neuronitis, hydrocephalus, brainstem infarctation, and internal carotid anomaly.
148 A Case of Papillary Endolymphatic Sac Tumor WON-SANG LEE, MD, and HO-KI LEE,MD, Seoul, Korea
146
Otolaryngologic Manifestations of Creutzfeldt-Jakob Disease--A Case Report: Creutzfeldt-Jakob Disease Presenting as Hearing Loss and Dizziness DOUGLAS C. BIGELOW, MD, DAVID E. SCHMIDT, MD, DAVID M. YEN, and GLEN W. KNOX, MD, Philadelphia, Pa.
Creutzfeldt-Jakob Disease (CJD) is a progressive fatal neurodegnerative disorder. One of the transmissible spongiform encephalopathies, this disease is caused by prions, an abnormal isoform of a normal host protein, and is unique in that it can occur as an inherited, sporadic, or acquired disease. Clinical findings provide the only clue to early stages of CJD. Because cerebellar abnormalities will develop in many of these patients, some may present to the otolaryngologist with dizziness. Hearing loss, however, is extremely rare. We describe a patient with CJD who presented with progressive hearing loss and subsequent dizziness. A review and discussion of the otolaryngologic manifestations of CJD is presented.
Papillary tumor of the endolymphatic sac is a very rare, silently aggressive neoplasm with extensive temporal bone destruction. A 34-year-old woman was seen with complaints of tinnitus and progressive hearing impairment in the right ear for 1 year and right facial weakness for 6 months. The eardrums were intact, but a puretone audiogram showed normal hearing in the left ear and a 60 dB sensorineural heating loss in the right ear. The speech discrimination score was 14%, and a caloric test revealed 35% canal paresis on the right ear. Right facial weakness was grade III according to the HouseBrackmann classification, and a 53% degenerative response was noted on electromyogram. Computerized tomography showed a retrolabyrinthine tumor with an irregular margin and prominent intratumoral bony spicules, and magnetic resonance imaging showed a heterogeneous mass with hyper-, hypo-, and isointense foci with T1 and T2 weighting. The tumor invaded the labyrinth and the mastoid portion of the fallopian canal. Angiography revealed that the tumor was hypervascular and was fed by ascending pharyngeal, occipi-
Otolaryngology Head and Neck Surgery Volume 113 Number 2
tal, and anterior inferior cerebellar arteries. Right-side mastoid exploration was done after embolization of the feeding vessels. Profuse bleeding was noted during dissection. The tumor contained fragile bone pieces and invaded the mastoid portion of the facial nerve and sigmoid sinus. The tumor was completely removed using a translabyrinthine and petrosal approach. The pathologic diagnosis was a papillary adenoma of endolymphatic sac. The patient is free of disease 5 months after treatment. 149
Eustachian Tube Obstruction (ETO) in Cats and Chinchillas: Preliminary Results MARCELO R. DETOLEDO PIZA, MD, PATRICIA A. SCHACHERN, MICHAEL M. PAPARELLA, MD, and SHINICHI SANO, MD, RibeirOo Preto, Brazil, and Minneapolis, Minn.
Of 18 cats and 27 chinchillas studied, bilateral eustachian tube obstruction (ETO) was performed in 15 cats and 23 chinchillas. Three cats and four chinchillas without obstruction or histologic evidence of otitis media were used as controis, and one obstructed animal from each species was excluded because of other pathologies. The animals were killed 2 days and 1, 2, and 4 weeks after surgery, respectively. After processing of the temporal bones, adjacent sections 20 Ix thick were stained for hematoxylin and eosin, alcian blue (pH 2.5), periodic acid-Schiff and alcian blue (pH 2.5)periodic acid-Schiff and studied under light microscopy. Quantitative and qualitative studies were done at specific areas of the protympanum, round window niche, and promontory in cats, and at the protympanum, round window niche, and apex of the cochlea in chinchillas. The gross histologic findings, number of goblet and ciliated cells, subepithelial space thickness, and staining characteristics of the goblet cells were studied. Our conclusions were as follows: (1) In the chinchilla the development of middle ear effusions was poor when compared with cats; (2) cats developed mucoid effusion, while the chinchilla developed serous or seropurulent effusions; (3) the inflammatory process was more generalized and intense in cats, affecting the middle ear mucosa as a whole, whereas in chinchillas it is mostly restricted to the protympanum area; (4) no granulation tissue was observed in the chinchilla, but new bone formation was common; (5) the total number of goblet cells in the chinchilla is much smaller than in cats, in normal and eustachian tube obstructed animals; (6) there was a tendency of the goblet cells to secrete more acid glycoproteins than neutral at the late stages of ETO in both species. The findings of this study demonstrate that the changes of the middle ear in cats with otitis media are more similar to those of humans than in the chinchilla. Furthermore, the cat is the species of choice for the study of mucoid otitis media.
Scientific Posters P203
150
The Auditory and Vestibular Manifestations of Charcot Marie-Tooth Disease DANIEL R. FEAR, MD, C. BRUCE MACDONALD, MD, and L. CLARKE COX, PHD, Boston, Mass.
Objective: To present the auditory-vestibular manifestations of Charcot Marie-Tooth Disease (CMT). Case Report: A 53-year-old woman complained of progressive imbalance over 1 year. Imbalance was described as the sense that she was about to fall to one side, was exacerbated by rapid head acceleration, and had no relation to postural changes. Symptoms were continuous, and she fell multiple times. She used a cane for 1 year, but for the last month required a walker. Bilateral tinnitus and heating loss developed over the last year. CMT had been diagnosed about 20 years earlier, with progressive distal limb weakness a n d wasting and peripheral sensory neuropathy. The neurotologic examination was normal except for the following: There was mild tremor in the left hand. Unsupported stance was unsteady, and she fell with eye closure. Vibrotactile sensation was reduced at the hands and ankles. Audiometry demonstrated a mild, somewhat asymmetric sensorineural hearing loss (SNHL). Auditory brainstem response showed poor waveform morphology bilaterally; however, the latencies for waves III and IV were within the normal range. Electronystagmography with rotary chair data showed hypofunction and reduced vestibulo-ocular gain, Ophthalmologic evaluation was refused. Discussion: CMT is an autosomal-dominant disorder manifested by progressive peripheral neuropathy, resulting in distal muscle atrophy and a peripheral sensory neuropathy. SNHL has been described in CMT. The Rosenberg-Chutorian Syndrome involves CMT with SNHL and optic atrophy. Other cranial neuropathies have also been described in CMT. Results: We describe a patient with CMT patient who had hearing loss and imbalance. The combination of vestibulopathy, sensory (proprioceptive) neuropathy, and muscle atrophy produced a devastating balance disorder. The literature on otologic manifestations of CMT is reviewed. 151
Facial Paralysis Following Otic Drop Usage SILVIO MARRA, MD, JOHN P. LEONETTI,MD, and SUSAN FOWLES, RN, Maywood, Ill.
Six days after undergoing a right superficial parotidectomy, a 59-year-old woman complained of ipsilateral otalgia. Although her parotid wound and facial movement appeared normal, the patient was noted to have otomycosis of the right external auditory meatus. After appropriate debridement, the patient was given acetic-boric Drops (Domeboro solution) and was instructed to return in 7 days. The patient returned in 48 hours with excruciating otalgia after placement of the drops and obvious incomplete facial paresis. This progressed to a total facial paralysis over the ensuing 2 days.