Ryoukichi Ikeda

A case of paranasal sinus lesions in IgG4-related sclerosing disease

In 2001, IgG4-related sclerosing disease was first reported in connection with autoimmune pancreatitis. In that report, clinical manifestations were apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, to date there has not been any report of the disease in the upper respiratory tract with the exception of only one description of a lesion in the pituitary gland that infiltrated the paranasal sinuses. We herein present a case of paranasal sinus lesions in IgG4related sclerosing disease. In a regular physical examination at her company, a 50-year-old woman was incidentally found to have right pleural effusion as revealed by chest x-ray films and was admitted to the Department of Respiratory Medicine at Tohoku Employees’ Pension Welfare Hospital. Serum IgE, IgG, IgA, and IgM levels were 118 mg/dL, 1349 mg/dL, 98 mg/dL, and 41 mg/dL, respectively; the serum levels of the IgG subclasses were as follows: IgG1, 685 mg/dL (normal 538-1244 mg/dL); IgG2, 402 mg/dL (normal 254-820 mg/ dL); IgG3, 104 mg/dL (normal 70 mg/dL); and IgG4, 258 mg/dL (normal 117 mg/dL). The serum level of soluble interleukin-2 receptor was 2420 U/mL, but levels of other serological makers, including antinuclear antibody, rheumatoid factor, anti-Sjögren’s syndrome (SS)-A antibody, or anti-SS-B antibody, could not be detected. Histological findings of the pleura corresponded to pleuritis. Immunostaining of the pleural specimen using a monoclonal antibody to IgG4 revealed numerous IgG4-positive plasma cells. The patient was referred to the otolaryngologic clinic with bloody rhinorrhea and postnasal drip. Paranasal CT revealed a soft tissue shadow in the left maxillary and ethmoid sinuses (Fig 1), the intensity of which was high in T1-weighted and low in T2-weighted MRI. Pathological specimens obtained from the maxillary sinus demonstrated submucosal infiltration of a number of lymphocytes and plasma cells with fibrosis (Fig 2A). Numerous IgG4-positive plasma cells were detected by immunostaining (Fig 2B). We diagnosed the present case as IgG4-related sclerosing disease in the paranasal sinus. She was treated with 30 mg/d of prednisolone, which resulted in marked clinical and laboratory improvement. The dose of prednisolone was gradually tapered, resulting in an IgG4 serum level of 78 mg/dL and a decrease in the right pleural effusion in chest x-ray films. After six months of treatment, she had no recurrence. The institutional review board of the Tohoku Employees’ Pension Welfare Hospital approved this study.

Management of patulous eustachian tube with habitual sniffing.

Objectives: Evaluation of the effect of conservative and surgical treatment in patients with patulous Eustachian tube (PET) associated with habitual sniffing. Study Design: Retrospective case review. Setting: University hospital otolaryngology department. Patients: Ninety-seven (23.4%) of 414 PET patients were found to have habitual sniffing to alleviate uncomfortable aural symptoms. Of these, 38 PET patients with sniffing habit were selected. Interventions: Conservative intervention included instructions to stop sniffing and nasal instillation of saline. Surgical interventions used ventilation tube (VT) insertion to the eardrum and/or transmyringeal insertion of the PET plug (PEP). Main Outcome Measures: Relief of uncomfortable symptoms and stopping sniffing. Results: In 52 (53.6%) of the 97 sniff-positive cases, retraction-type eardrum abnormalities or operated ear due to cholesteatoma were identified, whereas normal bilateral eardrums were observed in 34 (87.2%) of 39 sniff (−) cases. Abnormal findings in the eardrum were significantly more common in the sniff-positive group than in the sniff-negative group (p < 0.0001). Twenty-three patients (65.7%) stopped sniffing with conservative treatment. VT insertion was performed in 8 ears. Subsequent PEP was necessary in 2 of the 8 ears because of PET symptoms. PEP was successful in the other 11 ears, including one ear which needed additional VT insertion because of middle ear effusion. Conclusion: The management of habitual sniffing is difficult, but blocking the Eustachian tube by nasal instillation of saline and/or PEP could help PET patients to stop sniffing.

Glomangiopericytoma of the nasal cavity

Glomangiopericytoma is a rare tumor arising from the pericytes surrounding capillaries, and accounts for less than 0.5% of all sinonasal tumors. A 60-year-old male patient presented with a glomangiopericytoma of the nasal cavity manifesting as nasal obstruction and epistaxis, which was treated successfully with endoscopic excision. Histological examination showed multiplication of spindle-shaped to oval cells which reacted strongly to immunostaining for α-smooth muscle actin. Glomangiopericytoma is categorized as a borderline low malignancy tumor, which tends to recurrence. Strict follow-up is required, especially if complete resection is not achieved.

Risk factors for deterioration of bone conduction hearing in cases of labyrinthine fistula caused by middle ear cholesteatoma.

Objectives: We evaluated the risk factors and outcomes of bone conduction (BC) hearing in cases of labyrinthine fistulas treated under the basic principle of complete removal of the cholesteatoma matrix. Methods: A total of 47 patients with labyrinthine fistulas were analyzed. The fistulas were classified into smaller (no more than 3 mm) and larger fistulas (more than 3 mm). The fistulas were classified by depth into 3 stages. Preoperative symptoms and postoperative results with special reference to BC hearing were analyzed. Results: Total preoperative loss of BC hearing was found in 3 of 36 ears (9%) in the smaller-fistula group and 4 of 11 ears (36%) in the larger-fistula group; this was a statistically significant difference. The BC hearing was preserved after operation in 30 of 31 ears (97%) in the smaller-fistula group and 5 of 7 ears (71%) in the larger-fistula group; this difference was also significant. The stage (depth) of the fistula did not correlate with the postoperative BC hearing. Conclusions: In smaller labyrinthine fistulas, complete removal of the cholesteatoma matrix can be relatively safely performed. However, in patients with larger fistulas, there is a potential for a complete loss of BC hearing.

Relationship Between Clinical Test Results and Morphologic Severity Demonstrated by Sitting 3-D CT in Patients With Patulous Eustachian Tube.

Objective: To investigate the correlation of sitting 3-D computed tomography (CT) scans of the Eustachian tube (ET) with subjective and objective findings in patients with patulous Eustachian tube (PET). Study Design: Retrospective. Setting: Tertiary referral center. Subjects: A retrospective survey of medical records in Sen-En Hospital identified 40 patients and 62 ears with PET between September 2014 and June 2015. Method: Diagnosis of PET was based on the presence of three characteristic aural symptoms (autophony of voice or breathing sounds, and aural fullness), as well as verification of synchronous movement of the tympanic membrane in response to forced breathing under an endoscope. Any pressure changes in the external auditory canal (EAC) elicited by deep breathing and sniffing were detected by tubotympanoaerodynamography (TTAG). In addition, sonotubometry was performed where two parameters were used determined to evaluate ET function. Patients were examined by 3-D CT (Accuitomo; Morita, Kyoto, Japan) in the sitting position. The length of the closed ET lumen section was measured. Ears were divided into three groups as follows: completely open, closed-short (3 mm or less), and closed-long (longer than 3 mm). Results: The median length of the closed section of the ET lumen was 1.85 ± 2.69 mm in positive findings of PET. The three groups were significantly different in both aural fullness (p = 0.023) and, similarly, the difference in tympanic membrane movement (p = 0.032) among these three groups was also significantly different (p = 0.032). However, for autophony of breathing sounds, there was no significant difference with regard to autophony of breathing sounds among these three groups (p = 0.324). Although TTAG findings were did not reveal any significantly difference among these three groups (p = 0.589), the difference was significant (p = 0.001) in degree of EAC pressure change in TTAG. The difference among the three groups was significant (p = 0.001) based on sonotubometry findings. Conclusion: Under resting conditions, the lengths of the closed area of the ETs in PET groups are clearly shorter than in groups without PET based on sitting position CT scans in resting condition. Among the symptoms and clinical test findings including the ET function test results, the presence of tympanic membrane movement induced by respiration, the high degree of EAC pressure change in TTAG, as well as the positive results of sonotubometry are significantly correlated with the positive findings of sitting CT revealing the open ET.

Effectiveness of Kobayashi plug for 252 ears with chronic patulous Eustachian tube

Abstract Conclusions: Trans-tympanic plugging of the Eustachian tube (ET) with the silicone plug (Kobayashi Plug) induced long-term effectiveness for over 80% of chronic and severe patulous ET (PET) patients. The New Kobayashi Plug was more effective with fewer complication of plug descent to the pharyngeal orifice. Objective: To investigate the effectiveness and complications of trans-tympanic plugging of the ET using a Kobayashi Plug for chronic PET. Method: Trans-tympanic plugging of the ET using the Kobayashi Plug was performed for 252 ears of 191 patients. The Prototype Plug (115 ears of 82 patients in 2001–2007) and the New Plug (137 ears of 109 patients in 2008–2013) were inserted for chronic PET patients. Results: The success rate of the Kobayashi Plug for PET was 83.0% of a total (Prototype Plug 80.0%, New Plug 85.4%). In 26 ears, the Prototype Plugs were found to have descended toward the nasopharynx. Conversely, this did not happen with the New Plug. The rate of TM perforation (Prototype 22.6%, New 17.5%), middle ear effusion (Prototype 20.2%, New 10.2%) and ventilation tube placement (Prototype 14.8%, New 4.4%) decreased after transition to the New Plug.

Effect of Aspiration of Perilymph during Stapes Surgery on the Endocochlear Potential of Guinea Pig

Objective. Suction applied to the vestibule through the oval window during stapes surgery is considered a primary risk for postoperative sensorineural hearing impairment. This study investigated the mechanism of acute phase change in cochlear function caused by aspiration of the opened oval window. Study Design. Guinea pig model. Setting. Academic hospital laboratory. Subjects and Methods. Guinea pigs were divided into 3 groups: stapes footplate removed without suctioning (6 animals), with indirect suctioning (5 animals), and with direct suctioning of the vestibular perilymph (6 animals). Endocochlear potentials (EPs) were measured at the second turn of the cochlea, and temporal bones were examined histologically. Results. Removal of the stapes footplate without suctioning caused little change in the EP (original value, 80.12 ± 3.52 mV), indirect suctioning caused minor decline of the EP of 9.14 ± 1.84 mV, and partial recovery ensued, whereas direct but gentle suctioning, resulting in dry vestibule, caused reduction in the EP of 16.38 ± 6.63 mV. Recovery was not observed or incomplete. No animals showed profound decrease in the EP. Conclusion. Gentle suctioning and removal of the vestibular perilymph can cause a mild decrease in the EP even without damaging the inner ear structures. Therefore, suctioning of the perilymph should be avoided during stapes surgery because acute hearing loss can result even without damaging the inner ear structures. However, hearing loss may not be profound, if suctioning is not vigorous enough to cause damage to the inner ear structures.

Effect of vestibular labyrinth destruction on endocochlear potential and potassium concentration of the cochlea.

Partial labyrinthectomy can result in maintenance of hearing under certain circumstances, and the mechanism of the hearing impairment caused by labyrinthectomy is unclear. We hypothesized that disruption of the membranous labyrinth results in electrical leakage and electrolyte imbalance. This study investigated the change in cochlear function by measurement of endocochlear potential (EP) and potassium concentration ([K(+)]) caused by vestibular labyrinth destruction in the acute phase. Hartley guinea pigs underwent lateral semicircular canal (LSCC) transection with suctioning of the perilymph, ampullectomy, or destruction of the LSCC, superior SCC, and lateral part of the vestibule. The EP and [K(+)] were monitored using double-barreled ion-selective microelectrodes in the second turn of cochlea. The EP showed little to mild change after LSCC transectioning or ampullectomy, but declined variously and drastically after vestibulotomy. The EP did not recover but [K(+)] partially recovered after vestibulotomy. Disturbance of the mechanism of cochlear function caused by vestibular labyrinth destruction may involve reduction in the [K(+)] concentration in the endolymph.

Preventative effect of various fluids used in the epitympanic bulla on deterioration of cochlear function during labyrinthectomy

Abstract Conclusion: Preventing leakage of endolymph during vestibular destruction may be important in the acute phase. Objectives: Vestibulotomy causes significant decrease in the endocochlear potential (EP). The effect of artificial perilymph and distilled water administration during vestibulotomy on hearing preservation was evaluated in Hartley guinea pigs. Methods: Animals were divided into three groups: the epitympanic bulla was filled with artificial perilymph or distilled water, or was not filled with any solution (control group). The EP and [K+] were monitored using double-barrelled ion-selective microelectrodes in the second turn of the cochlea. Results: EP was not significantly different between the artificial perilymph and the distilled water groups, although both groups showed better preservation of EP compared with the control group. The [K+] was not significantly different between the three groups.

New Scoring System for Evaluating Patulous Eustachian Tube Patients.

OBJECTIVE To assess the efficacy of patulous Eustachian tube handicap inventory (PHI) for patulous Eustachian tube (PET) patients. STUDY DESIGN Prospective. SETTING Tertiary referral center. SUBJECTS A prospective survey of medical records in Sen-En Hospital identified 31 ears of 31 patients with definite PET who received insertion of the silicone plug as surgical treatment group, 29 ears of 29 patients treated with self-instillation of physiological saline solution as conservative treatment group, and 29 ears of 29 patients of sensorineural hearing loss without findings of PET treated between June 2015 and December 2015. METHOD Diagnosis of definite PET was based on the proposal on PET diagnosis criteria announced by the Otological Society of Japan. The evaluation scale of PHI was modified from the Japanese version of the tinnitus handicap inventory-12 (THI-12). The classification for grading of severity is defined as follows: 1) no handicap (0-8), 2) mild handicap (10-16), 3) moderate handicap (18-24), and 4) severe handicap (26-40), matching the severity grades of tinnitus handicap inventory-25 (THI-25). The outcome measurement was modified from the previous scoring system and is defined as 1) complete relief, 2) significant improvement, 3) slight improvement, 4) unchanged, and 5) worse, and is applied according to the classification for grading of severity. The PHI was conducted at the first visit to our center for all patients in the three groups. For cases requiring surgery for plug insertion, patulous Eustachian tube handicap inventory 10 (PHI 10) was also conducted postsurgery after the treatment (postsurgery). RESULTS The findings from questions 1 to 7 and 9 to 11 were significantly different between the surgical (presurgery) and conservative treatment groups (p < 0.05). Taking these results, we analyzed 10 questions excluding questions 8 and 12 (PHI 10). The total score of PHI 10 averaged 19.5 ± 9.3 (n = 31) and 30.6 ± 8.6 (n = 29) in the surgical treatment (presurgery) and conservative treatment groups, respectively, with a significant difference (p < 0.05). Internal consistency reliability testing of the PHI 10 yielded a Cronbach α of 0.887 for all questions. In the surgical treatment (presurgery) and conservative treatment groups, there were 0 (0%) and 3 cases (12%) of no handicap, 3 (10%) and 13 cases (50%) of mild handicap, 6 (19%) and 4 cases (15%) of moderate handicap, and 22 (71%) and 6 cases (23%) of severe handicap, respectively. There was a significant correlation between the PHI 10 and Likert scale (r = 0.796, p < 0.01). In the surgical group, the presurgery and postsurgery PHI 10 scores (n = 25) were 29.6 ± 8.5 and 7.8 ± 11.3, respectively. CONCLUSION The PHI 10 is suitable for evaluating severity of PET if the patients have been diagnosed as definite PET. Furthermore, this scoring system could be suitable for surgical treatment assessment.