Objective:To describe the radiologic dimensions of the Eustachian tube (pre-tympanic diameter, tubo-tympanic angle, and Eustachian tube angle) among normal ears, ears with chronic otitis media (COM) with and without cholesteatoma, in a sample of Filipino adults with COM; and to compare the dimensions of the three variables.

Methods:
Design:Retrospective Review of Records
Setting:Tertiary Government Training Hospital
Participants:The study included 69 CT scans of adults with COM, with images of 23 ears for each variable (normal ears, COM with and without cholesteatoma formation).

Results:In normal ears, the average pre-tympanic diameter was 3.976 ± 0.78 mm, the average tubo-tympanic angle was 41.529 ± 5.85 degrees, and the average Eustachian tube Angle (Reid Plane) was 27.077 ± 4.62 degrees. In patients with COM without cholesteatoma, the mean pre tympanic diameter was 4.002 ± 1.13 mm, the mean tubo-tympanic angle was 141.994 ± 6.30 degrees, and the mean measurement of the Reid plane was 24.649 ± 3.10 degrees. In ears with COM with cholesteatoma, the mean pre-tympanic diameter was 4.1993 ± 0.50mm, the mean tubo-tympanic angle was 138.789 ± 7.13 degrees, and the mean Reid plane was 26.483 ± 3.86 degrees. P-values for the pre-tympanic diameter, tubo-tympanic angle, and ET angle were .617, .198, and .098 respectively, indicating that there were no statistically significant differences in the ET dimensions of the ears with COM with cholesteatoma, without cholesteatoma and normal ears.

Conclusion:In our investigation, we found no statistically significant differences in Eustachian tube dimensions among individuals with normal ears and COM with or without cholesteatoma. This suggests that the Eustachian tube dimensions may not serve as a primary etiological factor for COM among our patients.

INTRODUCTION

Lamellar ichthyosis (LI) is an inherited rare disorder characterized by generalized scaling presenting at birth and persisting throughout life. It presents at birth with a collodion membrane, later developing into large, brown scales across the skin. LI requires ongoing treatment and monitoring due to physical and psychosocial impacts. This study highlights siblings who showed substantial quality-of-life improvements with oral retinoid therapy.

To present and discuss a detailed case summary, explore management options, and evaluate the treatment outcomes.

We report on a pair of siblings, a 24-year-old male and a 19-year-old female, who were born encased in a collodion membrane, and later presented with large, brown, plate-like scales all over the body. Skin changes were accompanied by intermittent heat intolerance and mild ectropion, which subsequently caused impaired quality of life while growing up. Histopathology results were consistent with lamellar ichthyosis. Both siblings responded well to oral Acitretin at 0.5 mglkglday, showing significant shedding of thick scales and a reduction of ectropion within the first two weeks of therapy.

Lamellar ichthyosis, a severe, lifelong disorder with psychosocial repercussions, requires long-standing, continual therapy. Maximizing treatment options with oral acitretin, addressing the psychosocial implications of the disease and getting patients actively involved in its management results in better treatment outcomes.

We report a case of a Filipino child who presented with yellowish hyperkeratotic plaques on the palms and soles with palmar transgredient extension to the wrists, a yellowish hyperkeratotic plaque over the coccygeal area, and brownish-black hyperkeratotic perianal plaques. Patient had delayed physical development and short stature, but no intellectual disability. Histopathologic examination showed palmoplantar keratoderma. These clinical findings of symmetrical palmoplantar keratoderma with periorificial keratotic plaques were consistent with Olmsted Syndrome. Oral retinoids with topical keratolytics afforded significant improvement with increased hand mobility. Although there is no curative management for these patients, current experimental therapies like epidermal growth factor receptor (EGFR) inhibitors and Transient Receptor Potential Vanilloid-3 (TRPV3) antagonists are promising. Olmsted Syndrome is a rare genodermatosis with 73 cases officially reported as of this writing. This is the first case to be reported from the Philippines.

Darier disease is an autosomal dominant manner, with complete penetrance and variable expressivity. The condition is caused by mutations in the ATP2A2 gene, which encodes SERCA2. Family history is often overlooked due to subtle clinical presentations and presentation is common at around 20 years of age.

Case Report:This is the case of a 79-year-old elderly female who presented with a three year history of intensely pruritic erythematous hyperkeratotic papules on the face, trunk, and all extremities aggravated by heat and sweating. She reported of similar skin lesions seen on her father and brother, however they were undiagnosed and not treated. Multiple medications, including Prednisone, topical steroids, and antibiotics, provided temporary relief. Skin punch biopsy was done which revealed Acantholytic acanthoma consistent with Darier’s disease. CBC, SGPT, SGOT, Alkaline Phosphatase, BUN, Creatinine, Lipid Profile, FBS were requested and she was managed with topical corticosteroids, isotretinoin, Urea 10% lotion, and oral anti-histamines.

Darier disease is an autosomal dominant genodermatosis caused by mutations in the ATP2A2 gene, often presenting during puberty with chronic symptoms like hyperkeratotic lesions and nail abnormalities. Diagnosis relies on clinical and histopathologic correlation, aided by family history, though variable expressivity can complicate it. Coexisting infections must be evaluated due to potential morbidity. Treatment options include systemic retinoids, immunomodulators, and topical therapies like retinoids and calcineurin inhibitors. Personalized therapies have shown promise. Patient education on trigger avoidance and genetic counseling is crucial for managing recurrence risk, while life expectancy remains comparable to the general population.

Lamellar Ichthyosis (LI) is a rare autosomal recessive disorder caused by mutations in genes, primarily TGM1, that affect skin barrier formation. It results in large, hyperpigmented, plate-like scales covering the entire body and persists throughout life. This case illustrates the chronic and debilitating nature of LI, highlights therapeutic approaches that improve quality of life, and emphasizes the importance of genetic testing in managing the condition.

Patient A, a 25-year-old female, and Patient B, a 22-year-old male, are Filipino siblings from non-consanguineous parents with no notable family history. Both were born encased in a collodion membrane that later revealed generalized erythema with large scales and deep fissures. As they aged, symptoms worsened, including reduced sweating, heat intolerance, ectropion, eclabium, cicatricial alopecia, palmoplantar hyperkeratosis, limited finger movement, and blurred vision from corneal scarring. Whole exome sequencing identified a homozygous pathogenic variant in the TGM1 gene (Arg396Cys). Their parents are carriers, giving future offspring a 25% risk of inheriting the condition. Acitretin therapy at 0.2 mg/kg/day, combined with topical keratolytics, led to significant improvement in symptoms and quality of life within four weeks.

Accurate diagnosis of ichthyosiform disorders requires thorough clinical documentation, family history, physical examination, and genetic findings. Effective management of lamellar ichthyosis needs a multidisciplinary approach, focusing on improving quality of life by addressing physical discomfort and social challenges. Genetic testing, especially Whole Exome Sequencing (WES), is crucial for precise diagnosis, genetic counseling, and informed family planning.

Darier disease is an autosomal dominant skin condition characterized histologically by acantholysis due to mutations in the ATP2A2 gene. This commonly presents in seborrheic distribution as greasy, yellow-brown keratotic papules. Presenting a case of a 14-year-old male, who experienced progressive red to brown scaly rashes which started on the face progressing to the trunk and inguinal areas in a seborrheic distribution with nail brittleness. Treatment included topical corticosteroids and retinoids, leading to gradual improvement. Management emphasizes the importance of avoiding triggers and highlighting the significance of early intervention and eventual multidisciplinary support.

BACKGROUND

Seborrheic keratosis is a common benign skin tumor treated with invasive methods like electrodessication, cryotherapy, or surgery. Topical therapy with trichloroacetic acid (TCA) may be a cheaper, non-invasive alternative with less complications. While TCA has been studied for other skin conditions, more research is needed on its use for seborrheic keratosis.

To compare the safety and efficacy of one time application of 80% TCA peel to 65% TCA peel in the clearance of raised seborrheic keratosis in Filipino patients.

One hundred one patients were randomized to either 80% or 65% TCA groups. Each participant underwent one session of treatment and was followed up after a month. Pre- and post-test size and thickness of lesions were compared between groups.

Based on Physician’s Lesion Assessment (PLA), both concentrations of TCA achieved high treatment success (TCA80: 66.7%, TCA65: 68%, p-value 0.89). A similar trend was observed based on Subject’s Self-Assessment (TCA80: 74%, TCA65: 74.5%, p-value 0.95). Similar reductions in lesion size and thickness were observed in both concentrations. In terms of safety, pain, pruritus, and erythema were mostly observed during and immediately after treatment, with little to no adverse events observed after 1 month in both groups.

One time application of either 80% or 65% TCA is effective in decreasing the size and thickness of seborrheic keratosis with little to no complications 1 month post- treatment. Both concentrations were safe, causing minimal and non-persistent pain, pruritus, and erythema immediately after application.

Delayed endolymphatic hydrops （DEH） is a rare disease that causes vertigo and is often misdiagnosed as other vertigo diseases. This article reports on a patient with vertigo who was easily misdiagnosed. The patient was a middle ear cholesteatoma complicated by labyrinthine fistula （LF）; however, his vertigo was episodic vertigo, which could not be explained solely by LF causing labyrinthitis. The possibility of endolymphatic hydrops was suspected, which was confirmed by inner ear magnetic resonance gadolinium imaging. This is the first reported case of middle ear cholesteatoma complicated by LF and DEH. The patient underwent surgical resection of the cholesteatoma and three semicircular canal obstructions at the same time. During two years postoperative follow-up, the patient did not experience a recurrence of vertigo. When diagnosing vertigo diseases, a careful history of vertigo is of utmost importance.
Humans ; Endolymphatic Hydrops/diagnosis* ; Cholesteatoma, Middle Ear/complications* ; Vertigo/complications* ; Labyrinth Diseases/complications* ; Magnetic Resonance Imaging/adverse effects* ; Semicircular Canals

Objective:For tympanosclerosis patients with ossicular chain fixation, we use ossicular chain bypass technique and evaluate its long-term effects. Methods:From June 2017 to June 2019, 147 patients with tympanosclerosis who underwent middle ear surgery with otoscopy in Yinchuan First People's Hospital were reviewed. The subjects were divided into three groups according to the implemented operation plan, 51 cases in the ossicular chain mobilization group（OCM）, 56 cases in the ossicular chain bypass reconstruction group（OCB）, and 40 cases in the malleus-incus complex resection reconstruction group（MICR）. Through a three-year follow-up, the medium and long-term effects of different operation plans were compared and analyzed. Results:There was no significant difference among the three groups in the incidence of tympanic membrane perforation, delayed facial nerve palsy, and the dispatch and displacement of PORP. The incidence of tympanic membrane retraction pocket or cholesteatoma after operation in OCB group（0） was significantly lower than that in OCM group（11.76%） and MICR group（7.5%）（P<0.05）. At 12 months after operation, ΔABG of OCB group and MICR group were better than that in the OCM group（P<0.05）. At 36 months after operation, ΔABG of OCB group was better than that in the OCM group（P<0.05）, and there was no significant difference between OCB group and MICR group. The audiological performance of patients with epitympanic sclerosis（ETS） at 12, 24 and 36 months after operation was better than that of patients with posterior tympanosclerosis（PTS） and total tympanosclerosis（TTS）（P<0.05）. Conclusion:Compared with patients undergoing ossicular chain mobilization and malleus-incus complex resection for ossicular chain reconstruction, patients with tympanosclerosis undergoing bypass technique have better and stable hearing prognosis in medium and long term. This technique can effectively prevent the formation of retracted pocket and cholesteatoma in patients with tympanosclerosis after operation.
Humans ; Tympanosclerosis ; Ear Ossicles/surgery* ; Ear, Middle ; Malleus/surgery* ; Cholesteatoma ; Retrospective Studies ; Ossicular Prosthesis ; Treatment Outcome

Objective:To analyze the clinical diagnosis, treatment ，and surgical timing of otogenic intracranial complications. Methods:The clinical data of 11 patients with intracranial complications with ear symptoms as the first manifestation in Department of Otorhinolaryngology Head and Neck Surgery, Qilu Hospital of Shandong University（Qingdao） from December 2014 to June 2022 were collected, including 8 males and 3 females, aged from 4 to 69 years. All patients had complete otoendoscopy, audiology, imaging and etiology examination, and the diagnosis and treatment plan was jointly developed through multidisciplinary consultation according to the critical degree of clinical symptoms and imaging changes. Among the 11 patients, 5 cases were treated with intracranial lesions first in neurosurgery department and middle ear lesions later in otolaryngology, 3 cases of meningitis, were treated with middle ear surgery after intracranial infection control, 1 case was treated with middle ear lesions and intracranial infection simultaneously, and 2 cases were treated with sigmoid sinus and transverse sinus thrombosis conservatively. They were followed up for 1-6 years. Descriptive statistical methods were used for analysis. Results:All the 11 patients had ear varying symptoms, including ear pain, pus discharge and hearing loss, etc, and then fever appeared, headache, disturbance of consciousness, facial paralysis and other intracranial complication. Otoendoscopy showed perforation of the relaxation of the tympanic membrane in 5 cases, major perforation of the tension in 3 cases, neoplasia in the ear canal in 1 case, bulging of the tympanic membrane in 1 case, and turbidity of the tympanic membrane in 1 case. There were 4 cases of conductive hearing loss, 4 cases of mixed hearing loss and 3 cases of total deafness. Imaging examination showed cholesteatoma of the middle ear complicated with temporal lobe brain abscess in 4 cases, cerebellar abscess in 2 cases, cholesteatoma of the middle ear complicated with intracranial infection in 3 cases, and sigmoid sinus thrombophlebitis in 2 cases. In the etiological examination, 2 cases of Streptococcus pneumoniae were cultured in the pus of brain abscess and cerebrospinal fluid, and 1 case was cultured in streptococcus vestibularis, Bacteroides uniformis and Proteus mirabilis respectively. During the follow-up, 1 patient died of cardiovascular disease 3 years after discharge, and the remaining 10 patients survived. There was no recurrence of intracranial and middle ear lesions. Sigmoid sinus and transverse sinus thrombosis were significantly improved. Conclusion:Brain abscess, intracranial infection and thrombophlebitis are the most common otogenic intracranial complications, and cholesteatoma of middle ear is the most common primary disease. Timely diagnosis, multidisciplinary collaboration, accurate grasp of the timing in the treatment of primary focal and complications have improved the cure rate of the disease.
Female ; Humans ; Male ; Brain Abscess/therapy* ; Cholesteatoma ; Deafness/etiology* ; Hearing Loss/etiology* ; Lateral Sinus Thrombosis/therapy* ; Retrospective Studies ; Thrombophlebitis/therapy* ; Child, Preschool ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Cholesteatoma, Middle Ear/therapy* ; Central Nervous System Infections/therapy* ; Sinus Thrombosis, Intracranial/therapy* ; Ear Diseases/therapy*